February 2023 Medical Image of the Month: Reversed Halo Sign in the Setting of a Neutropenic Patient with Angioinvasive Pulmonary Zygomycosis

Figure 1. Axial reconstructions from unenhanced (A) and enhanced (B) chest CTs performed 1 week prior to admission (A) and at admission (B) demonstrating rapid interval increase in size of an initially small left upper lobe nodule (arrow) with extensive central necrosis manifesting as a “reversed halo” sign (circled, B).

Figure 2. Sagittal reconstructions from unenhanced (A, C) and enhanced (B) chest CTs through the left lung performed 1 week prior to admission (A), at admission (B), and 2 weeks after admission (C). Small nodules on initial CT (arrows, A) rapidly grow with prominent central necrosis (circle, B). The follow up CT after the patient started improving demonstrates an “air crescent” sign (arrowhead, C) consistent with improving angioinvasive fungal infection.

Figure 3. Low power view, GMS special stain (A) demonstrating a pulmonary artery with fungal elements invading into the wall and out into the surrounding lung parenchyma. There are variable and broad hyphae, with rare septation, many short fragments compatible with Rhizopus species grown in fungal culture. Low power view, H & E stain (B) from a different portion of the sample demonstrating fungal hyphae and spores with thinner morphology, right angle-branching, and calcium oxalate crystals, morphologically compatible with Aspergillus. This may represent secondary colonization of damaged lung.

A 66-year-old man presented to our emergency department with fever and lethargy. A CBC demonstrated profound neutropenia with an absolute neutrophil count of <0.50x10⁹ cells/L (critically low). The patient was admitted and workup for febrile neutropenia was begun. The patient’s past medical history includes CLL (recently confirmed to be in remission by bone marrow biopsy), hypogammaglobulinemia/capillary leak syndrome (presumably related to obinutuzumab therapy, for which patient receives monthly IVIG), and coccidioidomycosis (for which the patient has been followed by infectious disease at our institution, is on fluconazole). An outpatient chest CT performed 1 week prior to presentation to follow up pulmonary nodules demonstrated a few scattered small, but new, inflammatory-appearing nodules (Figure 1A, 2A).

A repeat chest CT was performed at time of admission, 7 days after the initial CT, which demonstrated marked interval increase in size of the small nodules, now represented as large areas of mass-like consolidation including a large finding in the left upper lobe displaying a reversed-halo sign (Figure 1B, 2B). Rapidly progressive fungal infection in the setting of neutropenia was favored. Due to rapid clinical deterioration and development of sites of infection outside the lungs, the decision was made to resect the left upper lobe for source control. The patient tolerated the procedure well, pathology from the specimen demonstrated pulmonary angioinvasive zygomycosis (mucormycosis) with broad areas of hemorrhagic pulmonary infarction, neutrophilic infiltrates and organizing hemorrhagic pneumonia. There were many invasive fungal organisms extending through the infarcted lung tissue. A culture of the lung showed Rhizopus species. There was prominent fungal angioinvasion with thrombosis in and around the infarcted lung. There were additional fungi in a bronchus that were thinner with more spores, septations, and elaborating oxalate crystals that were more consistent with Aspergillus species suggesting polymicrobial fungal infection. The patient was started on amphotericin B and posaconazole as well as filmgastrin. His neutropenia slowly improved, as did his clinical situation. A follow-up CT performed  2 weeks later demonstrated an air-crescent sign in the left lower lobe consistent with improving angioinvasive fungal infection in the setting of resolving neutropenia (Figure 2C). 

The reversed halo sign consists of a finding of peripheral consolidation and central ground glass, in counter distinction to the CT halo sign, which consists of a nodule or mass (or mass-like consolidation) surrounded by ground glass (1). Interestingly, the halo sign was initially described in the setting of angioinvasive aspergillus infection (2), yet the opposite “reversed halo” sign is, in this case and many other cases, also described in the setting of invasive pulmonary fungal infection (3). The reversed halo sign was classically described in the setting of cryptogenic organizing pneumonia (4), where there is central disease clearing. This sign is also described as the “atoll” sign (5), representing relatively normal, improving lung in that situation. In the setting of invasive fungal infection, the central ground glass represents the opposite situation: dead, necrotic lung rather than improving lung. Although organizing pneumonia and invasive fungal infection are well-recognized causes of the reversed halo sign, the sign is by no means specific. Reversed halo signs can be seen in a wide variety of pathologies including paracoccidioidomycosis, pneumocystis pneumonia, tuberculosis, community-acquired pneumonia, lymphomatoid granulomatosis, granulomatosis with polyangiitis, lipoid pneumonia, sarcoidosis, pulmonary infarction, post-radiofrequency ablation and more (6).

Clinton Jokerst MD¹, Yasmeen Butt MD², Ann McCullough MD², Carlos Rojas MD¹, Prasad Panse MD¹, Kris Cummings MD¹, Eric Jensen MD¹ and Michael Gotway MD¹

Departments of Radiology¹

Mayo Clinic Arizona, Scottsdale, AZ USA

Departments of Pathology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008 Mar;246(3):697-722. [CrossRef] [PubMed]
2. Kuhlman JE, Fishman EK, Siegelman SS. Invasive pulmonary aspergillosis in acute leukemia: characteristic findings on CT, the CT halo sign, and the role of CT in early diagnosis. Radiology. 1985 Dec;157(3):611-4. [CrossRef] [PubMed]
3. Wahba H, Truong MT, Lei X, Kontoyiannis DP, Marom EM. Reversed halo sign in invasive pulmonary fungal infections. Clin Infect Dis. 2008 Jun 1;46(11):1733-7. [CrossRef] [PubMed]
4. Kim SJ, Lee KS, Ryu YH, Yoon YC, Choe KO, Kim TS, Sung KJ. Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR Am J Roentgenol. 2003 May;180(5):1251-4. [CrossRef] [PubMed]
5. Zompatori M, Poletti V, Battista G, Diegoli M. Bronchiolitis obliterans with organizing pneumonia (BOOP), presenting as a ring-shaped opacity at HRCT (the atoll sign). A case report. Radiol Med. 1999 Apr;97(4):308-10. [PubMed]
6. Godoy MC, Viswanathan C, Marchiori E, Truong MT, Benveniste MF, Rossi S, Marom EM. The reversed halo sign: update and differential diagnosis. Br J Radiol. 2012 Sep;85(1017):1226-35. [CrossRef] [PubMed]

Cite as: Jokerst C, Butt Y, McCullough A, Rojas C, Panse P, Cummings K, Jensen E, Gotway M. February 2023 Medical Image of the Month: Reversed Halo Sign in the Setting of a Neutropenic Patient with Angioinvasive Pulmonary Zygomycosis. Southwest J Pulm Crit Care Sleep. 2023;26(2):21-23. doi: https://doi.org/10.13175/swjpccs003-23 PDF

Medical Image of the Month: Metastatic Spindle Cell Carcinoma of the Breast

Figure 1. A coronal reconstruction of the patient’s CT of the chest with contrast in lung windows demonstrates extensive, bilateral metastatic disease, with the greatest burden of disease situated in the right lower lobe. Areas of cystic change (blue arrows) and cavitary disease (red arrow) are present.

Clinical Scenario: A 71-year-old woman with primary malignancy of the breast in remission post bilateral mastectomy in 2005 and 2008, presented to the emergency room with progressive shortness of breath for the past 6 months. Upon arrival to the emergency room, she described localized sharp chest pain along the right thoracic wall which had gradually worsened over the past three months. The pain was exacerbated with movement and with deep inspiration. She also endorsed significant hemoptysis, expectorating approximately 500 ml of bloody sputum on the morning of her presentation. Pertinent vitals revealed that she was both tachycardic and tachypneic, saturating 94% on room air with an increased work of breathing. Physical examination was significant for coarse breath sounds and diminished right sided lung sounds. Initial labs demonstrated a normal troponin and an unremarkable EKG. A chest radiograph demonstrated a large left mediastinal and hilar mass with numerous parenchymal nodules bilaterally. A CT of the chest with contrast (Figure 1) demonstrated widespread lung nodules, most notably in the right lung with a confluent mass in the right base. No significant focal lesions were seen in the chest wall or breast regions. A biopsy of the left mediastinal mass was performed and confirmed metastatic spindle cell carcinoma originating from her primary breast cancer.

Discussion: Spindle cell carcinoma of the breast, a variant classified under metaplastic carcinoma, is a rare entity occurring in less than 1% of all incidences of primary breast cancer, and most commonly seen in postmenopausal women (2,5). Treatment is primarily surgical resection. The role of radiation and chemotherapy is unclear and varies per patient treatment plan. Tumors are typically triple-negative, limiting therapeutic options (1,2,5). Primary tumor diameter and grade may be the most important prognostic factors, although prognosis regarding spindle cell carcinoma is generally poor. At the time of diagnosis, incidence of axillary lymph node metastasis was 40 – 56% with a high grade of recurrence at 57% - 63% (1,4,5). Most common extra nodal metastasis was to the lungs. In addition to the poor prognosis of spindle cell carcinomas and high rates of local recurrence, metastatic disease is also frequently seeing in patients, such as the metastatic disease seen with our patient (2-4).

Our patient’s primary breast cancer was treated with a bilateral mastectomy followed by neither chemotherapy nor radiation therapy based on patient’s preference and discussion with her oncology team. The primary tumor was in the left breast with recurrence to the right breast and metastasis to bilateral lungs. Like other cases, a biopsy revealed a triple-negative tumor. She was discharged on supplemental oxygen and is expected to receive P13K inhibitor therapy for targeted palliative treatment.

Vinita Kusupati MD, MBA and Stefano Natali DO

Department of Internal Medicine,

Banner University Medical Center-Tucson Campus

Tucson, AZ USA

References

1. Adem C, Reynolds C, Ingle JN, Nascimento AG. Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature. Br J Cancer. 2004 Jul 19;91(2):237-41. [CrossRef] [PubMed]
2. Alaoui M'hamdi H, Abbad F, Rais H, Asmouki H, Soumani A, Khouchani M, Belbaraka R. Rare variant of metaplastic carcinoma of the breast: a case report and review of the literature. J Med Case Rep. 2018 Feb 21;12(1):43. [CrossRef] [PubMed]
3. Carter MR, Hornick JL, Lester S, Fletcher CD. Spindle cell (sarcomatoid) carcinoma of the breast: a clinicopathologic and immunohistochemical analysis of 29 cases. Am J Surg Pathol. 2006 Mar;30(3):300-9. [CrossRef] [PubMed]
4. Khan HN, Wyld L, Dunne B, Lee AH, Pinder SE, Evans AJ, Robertson JF. Spindle cell carcinoma of the breast: a case series of a rare histological subtype. Eur J Surg Oncol. 2003 Sep;29(7):600-3. [CrossRef] [PubMed]
5. Tse GM, Tan PH, Putti TC, Lui PC, Chaiwun B, Law BK. Metaplastic carcinoma of the breast: a clinicopathological review. J Clin Pathol. 2006 Oct;59(10):1079-83. [CrossRef] [PubMed]
6. Tse GM, Tan PH, Lui PC, Putti TC. Spindle cell lesions of the breast--the pathologic differential diagnosis. Breast Cancer Res Treat. 2008 May;109(2):199-207. [CrossRef] [PubMed]

Cite as: Kusupati V, Natali S. Medical image of the month: metastatic spindle cell carcinoma of the breast. Southwest J Pulm Crit Care. 2021;22(6):114-5. doi: https://doi.org/10.13175/swjpcc021-21 PDF 

March 2018 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Imaging Case of the Month CME Information  

Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity.

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Michael B. Gotway, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity. 

Learning Objectives: As a result of completing this activity, participants will be better able to:

1. Interpret and identify clinical practices supported by the highest quality available evidence.
2. Establish the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Translate the most current clinical information into the delivery of high quality care for patients.
4. Integrate new treatment options for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at the Arizona Health Sciences Center.

Current Approval Period: January 1, 2017-December 31, 2018

Clinical History: A 56-year-old woman with no significant past medical history underwent routine breast imaging (MRI) which showed an abnormality outside the breast (images not shown). She has a sister with recently-diagnosed breast malignancy. The patient smoked for 30 years, quitting 10 years ago. Her surgical history is remarkable only for a tubal ligation and hysterectomy, and she is asymptomatic. Her medications consist only of vitamins and supplements.

Laboratory evaluation showed a normal complete blood count, electrolyte panel, and liver function tests. Frontal and lateral chest radiography (Figure 1) was performed.

Figure 1. Frontal (A) and lateral (B) chest imaging.

Which of the following represents the most accurate assessment of the frontal chest imaging findings? (Click on the correct answer to proceed to the second of ten pages)

1. Chest frontal imaging shows a focal right lung nodule
2. Chest frontal imaging shows basilar fibrosis
3. Chest frontal imaging shows mediastinal and peribronchial lymphadenopathy
4. Chest frontal imaging shows multiple, bilateral small nodules
5. Chest frontal imaging shows normal findings

Cite as: Gotway MB. March 2018 imaging case of the month. Southwest J Pulm Crit Care. 2018;16(3):126-37. doi: https://doi.org/10.13175/swjpcc041-18 PDF 

January 2018 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Clinical History: An 81-year-old woman with little significant past medical history complained of a dry cough for the previous 1.5 years, but without hemoptysis or shortness of breath. The patient’s past medical history was remarkable only for hypothyroidism, for which she was taking levothyroxine. She smoked for 1 year only, at age 19. Her past surgical history was negative and she denied any alcohol use. Her only other medications included vitamin D3 supplementation and over-the-counter cough medicine.

Physical examination was remarkable only for coarse, left-greater-than-right basal rales. The patient’s oxygen saturation was 98% on room air. The patient’s vital signs were within normal limits and she was afebrile.

Laboratory evaluation showed a normal complete blood count, electrolyte panel, and liver function tests. Frontal chest radiography (Figure 1) was performed.

Figure 1. Frontal (A) and lateral (B) chest radiography.

Which of the following represents the most accurate assessment of the chest radiographic findings? (Click on the correct answer to proceed to the second of eleven pages)

1. Chest radiography shows basilar fibrosis
2. Chest radiography shows left lower lobe consolidation
3. Chest radiography shows mediastinal and peribronchial lymphadenopathy
4. Chest radiography shows multiple small nodules
5. Chest radiography shows normal findings

Cite as: Gotway MB. January 2018 imaging case of the month. Southwest J Pulm Crit Care. 2018;16(1):16-27. doi: https://doi.org/10.13175/swjpcc001-18 PDF 

Medical Image of the Week: Pulmonary Infarction- the “Reverse Halo Sign”

Figure 1. CT Chest with contrast. Two different levels in the same patient displayed on mediastinal windows. Several triangular shaped subpleural lesions with annular peripheral solid appearance are depicted, better characterized in the lung windows below (yellow arrows). Note the partial filling defect (red arrow on B), indicating a non-occlusive thrombus(arrow). Bilateral pleural effusions are also identified.

Figure 2. CT Chest with contrast, lung window corresponding to the levels in Figure 1 above. Note the triangular shaped subpleural lesions with peripheral solid appearance and ground glass center, characteristic of the atoll sign (arrows). As above, bilateral pleural effusions are present.

Pulmonary infarction is a known complication of pulmonary embolism (PE), a common disorder that results in 100,000-200,000 deaths annually in the United States. Computed tomography (CT) is the first-line modality to assess the pulmonary circulation with the ability to directly the visualize pulmonary emboli as well as pleuro-parenchymal abnormalities.

The appearance of a pulmonary infarct varies depending on the degree of ischemic injury in the setting of a dual blood supply to the lung. Infarcts occur more commonly in the periphery of the lung, given, the alternate blood supply by the bronchial arteries, is not as efficient as it is centrally. This location is also favored by the more common occurrence after occlusion of small peripheral arteries of 3 mm or less in caliber.

On CT lung infarcts can take the can take the “reverse halo” sign, also known as the “atoll” sign configuration, representing a focal area of decreased enhancement, and surrounding solid appearance. In the case of lung infarcts, the lesions typically have a broad pleural base triangular form with apex toward the hilum (1). Pathologically this corresponds to a hemorrhagic consolidation. The center of the lesion appears to correspond to aerated non-infarcted lung coexisting side by side with infarcted lung in the same lobule. The broad-based configuration is explained by the fan shaped distribution of the arteries as they extend out into the periphery. The convex border reflects the extravasated blood within the infarcted lung. Once the hemorrhage reabsorbs, the infarct heals completely or may leave behind a linear band of scarring.

From the imaging stand point, the reverse halo sign initially described in cryptogenic organizing pneumonia, has also been noted in patients with fungal disease, granulomatosis with polyangiitis, sarcoidosis and neoplastic disease among others (2).

George R Wu MS IV¹, Berndt Schmit MD², Veronica Arteaga MD², and Diana Palacio MD²

¹Arizona College of Osteopathic Medicine, Midwestern University, Glendale, AZ USA

²Division of Thoracic Imaging, University of Arizona, Tucson, AZ USA

References

1. He H, Stein MW, Zalta B, Haramati LB. Pulmonary infarction: spectrum of findings on multidetector helical CT. J Thorac Imaging. 2006;21(1):1-7. [CrossRef] [PubMed]
2. Godoy MC, Viswanathan C, Marchiori E, et al. The reversed halo sign: update and differential diagnosis. Br J Radiol. 2012;85(1017):1226-35. [CrossRef] [PubMed]

Cite as: Wu GR, Schmit B, Arteaga V, Palacio D. Medical image of the week: pulmonary infarction- the “reverse halo sign”. Southwest J Pulm Crit Care. 2017;15(4):162-3. doi: https://doi.org/10.13175/swjpcc124-17 PDF 

Medical Image of the Week: Tortuosity of Thoracic Aorta Mimicking a Lung Mass

Figure 1. PA (A) and lateral (B) chest X-ray showing a 5x4 cm round mass with sharp margins in retrocardiac area.

Figure 2. A-C: Initial CT image showing thoracic aorta acutely angulated above the diaphragm and crossing to the right side of the chest. Then the aorta acutely angulates again and descends into the abdomen on the right. D: Follow-up CT image after 2 years showing saccular dilatation of transverse area of thoracic aorta.

An 83-year-old female presented with epigastric discomfort and nausea for 1 month. Her past medical history included hypertension and osteoarthritis. Her vital signs at were unremarkable. Her electrocardiogram revealed only atrial premature beats. Laboratory examination, including complete blood count, liver function test, blood urea nitrogen, creatinine, and electrolytes were normal.

Esophagogastroduodenoscopy revealed minimal changes of reflux esophagitis, erosive gastritis, and extrinsic compression of lower esophagus. Her chest x-ray (Figure 1) showed a 5x4 cm sized round retrocardiac mass with sharp margin. Chest CT was ordered to evaluate the lung mass and it revealed that acutely angulated lower thoracic aorta which crossed from left to right above the left diaphragm (Figure 2). After treatment with a proton pump inhibitor and a gastrointestinal pro-motility agent, her symptoms gradually decreased. Follow-up CT after 2 years shows saccular dilatation of the transverse area of thoracic aorta (Figure 2D), however, she has no specific symptoms.

Abnormal vascular structures like a severe tortuous thoracic aorta occasionally can be confused with a lung mass or neoplasm. The most common cause of aortic disease mimicking lung mass on CXR is an aortic aneurysm (1). Some cases have reported an intervention or even an operation being performed. The symptoms of tortuosity of thoracic aorta are varied from asymptomatic to dysphagia, gastroesophageal reflux, nausea and vomiting (2). Therefore, clinical symptom is not helpful to diagnose the underlying cause. As in this case, chest computed tomography (CT) can be beneficial for the differential diagnosis between vascular lesion and lung mass. Chest CT also gives additional information for communication of the aneurysm with the aorta, relationship of vascular structure to mediastinal organs. In children, Loeys-Dietz syndrome or arterial tortuosity syndrome should be considered (3). If aortic aneurysm or tortuosity of aorta is diagnosed as a cause in older age, close observation should be performed because of the possibility of progression to aortic aneurysm, dissection or compression of adjacent organs.

Jong Seol Park, MD and Yong Sung Kim, MD, PhD

Department of Internal Medicine

Wonkwang University Sanbon Hospital

Gunpo, Korea

References

1. Wixson D, Baltaxe HA, Sos TA. Pitfalls in the plain film evaluation of the thoracic aorta: the mimicry of aneurysms and adjacent masses and the value of aortography. Part I. Transverse aortic arch. Cardiovasc Radiol. 1979 Apr 27;2(2):69-76. [CrossRef] [PubMed]
2. Badila E, Bartos D, Balahura C, Daraban AM. A rare cause of Dysphagia - Dysphagia aortica - complicated with intravascular disseminated coagulopathy. Maedica (Buchar). 2014 Mar;9(1):83-7. [PubMed]
3. Na KJ, Park KH. Multiple aortic operations in loeys-dietz syndrome: report of 2 cases. Korean J Thorac Cardiovasc Surg. 2014 Dec;47(6):536-40. [CrossRef] [PubMed] 

Cite as: Park JS, Kim YS. Medical image of the week: tortuosity of thoracic aorta mimicking a lung mass. Southwest J Pulm Crit Care. 2017;15(2):80-1. doi: https://doi.org/10.13175/swjpcc086-17 PDF 

July 2017 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, Arizona USA

Clinical History: A 56-year-old man with no significant past medical history presented with complaints of cough, shortness of breath, and productive sputum. Frontal and lateral chest radiography (Figure 1) was performed.

Figure 1. Frontal (A) and lateral (B) chest radiography.

Which of the following statements regarding the chest radiograph is most accurate? (Click on the corect answer to proceed to the second of nine pages)

1. The chest radiograph shows a diffuse linear, interstitial pattern
2. The chest radiograph shows a large pleural effusion
3. The chest radiograph shows a mediastinal mass
4. The chest radiograph shows numerous small nodules
5. The chest radiograph shows right lower lobe consolidation

Cite as: Gotway MB. July 2017 imaging case of the month. Southwest J Pulm Crit Care. 2017;15(1):17-27. doi: https://doi.org/10.13175/swjpcc090-17 PDF 

Medical Image of The Week: Urothelial Carcinoma with Pulmonary Metastases Presenting with Shoulder Pain

Figure 1. AP view of the left shoulder demonstrated multiple pulmonary nodules.

Figure 2. Coronal view of chest CT demonstrating innumerable pulmonary nodules with thick walled cavitations.

Figure 3. Axial view of chest CT demonstrating innumerable pulmonary nodules with thick walled cavitations.

A 68 year old man with a past medical history significant only for mild hyperlipidemia and distant cigar smoking presented to this primary physician’s office with a chief complaint of left sided shoulder pain for more than 6 months duration. His only other complaint was a hacking morning cough that was attributed to GERD after resolution with omperazole therapy. He was without any other complaints such as weight loss, fevers, chills, night sweats, shortness of breath, or dyspnea on exertion. His physical exam was without any abnormality. An initial radiograph of the rileft shoudler was obtained which was without any obvious bony abnormality but demonstrated numerous potential pulmonary nodules (Figure 1).  He was then referred to pulmonology for further assessment. A chest CT scan peformed with contrast again demonstrated numerous pulmonary nodules with thick walled central cavitations throughout the lung parenchyma bilaterally (Figures 2 & 3). Additional testing performed included Coccidioides serologies, c-ANCA, p-ANCA, Quantiferon Gold, PSA, and rheumatoid arthritis serology (RF/CCP) all of which were negative. He was taken for a CT guided lung biopsy of one of the nodules and the biopsy result demonstrated a poorly-differentiated carcinoma with focal squamous differential; nuclear “salt and pepper” features; along with immunostaining consistent with poorly differentiated urothelial cell carcinoma. The patient was referred to oncology but refused potential palliative chemotherapy.

The differential diagnoses for cystic and cavitary lung disease is very broad, therefore it is of utmost importance to differentiate between cystic and cavitary diseases. Typically, cystic lung diseases are round parenchymal lucencies with a thin wall, typically <2mm in thickness, whereas cavitary lung disease are round luciencies typically with a wall >4mm in thickness, but overlapp between cystic and cavitary lung disease does exist (1,2). Without evidence or symptomology to suggest malignancy, initial differential diagnosis must include infectious causes of cystic/cavitating lung disease. In regions such as the Southwestern United States where diseases such as Coccidioidomycosis is endemic, this must be included in the differential diagnosis, as does other potential infectious cystic/cavitating lung disease such as M. tuberculosis, Pneumocystis infection, or Klebsiella infection (2). Granulomatosis with polyangiitis (Wegener’s granulomatosis), as well as other rheumatologic conditions must also be included in the initial differential diagnosis. In this case, infectious and rheumatologic testing was negative. Biopsy was then necessary to determine etiology which was consistent with a metastatic urothelial carcinoma. A CT urogram was performed which was without evidence of primary tumor. Literature review suggests that approximately 65% of metastatic urothelial cancers metastasize to the lung, and often form nodules with central necrosing cavitations (3).

Benjamin Jarrett MD, MPH¹, Huthayfa Ateeli, MBBS², Harbhajan Singh, MD²

¹Department of Internal Medicine and ²Department of Pulmonary and Critical Care Medicine

University of Arizona College of Medicine and Southern Arizona VA Healthcare System

Tucson, Arizona USA

References

1. Raoof S, Bondalapati P, Vydyula R, et al. Cystic lung diseases: algorithmic approach. Chest. 2016 Oct;150(4):945-65. [CrossRef] [PubMed]
2. Gadkowski LB, Stout JE. Cavitary pulmonary disease. Clin Microbiol Rev. 2008 Apr;21(2):305-33. [CrossRef] [PubMed]
3. Shinagare AB, Fennessy FM, Ramaiya NH, Jagannathan JP, Taplin ME, Van den Abbeele AD. Urothelial cancers of the upper urinary tract: metastatic pattern and its correlation with tumor histopathology and location. J Comput Assist Tomogr. 2011 Mar-Apr;35(2):217-22. [CrossRef] [PubMed]

Cite as: Jarrett B, Ateeli H, Singh H. Medical image of the week: urothelial carcinoma with pulmonary metastases presenting with shoulder pain. Southwest J Pulm Crit Care. 2017;14(6):315-7. doi: https://doi.org/10.13175/swjpcc067-17 PDF 

Medical Image of the Week: Spontaneous Pneumothorax in End Stage Fibrotic Lung Disease

Figure 1. Chest x-ray showing moderate-sized right pneumothorax with a pigtail chest tube in place, diffuse reticular interstitial opacities.

Figure 2. Chest CT showing extensive centrilobular emphysema, moderate right pneumothorax with pigtail chest drain on the right, subpleural reticular opacities with peripheral and basilar preponderance suggesting interstitial fibrotic lung disease, and diffuse lung cysts - heterogenous in size.

A 61-year-old nonsmoking man with chronic obstructive lung disease, pulmonary hypertension, pulmonary fibrosis, hypertension, coronary artery disease with congestive heart failure, presented with recurrent pneumothorax, pneumomediastinum, extensive subcutaneous emphysema and bronchopleural fistula.

The patient reported ongoing symptoms of exertional dyspnea, fatigue, and coughing for years. His environmental exposures were notable for exposure to birds since early childhood. He had 6 cockatiels and 2 doves living inside his home and is directly responsible for their care. Former occupational exposures include painting and sandblasting. Family history was notable for early onset non specified lung disease in his father, and rheumatoid arthritis in his mother.

Lung function testing performed prior to the bronchopleural fistula revealed moderate obstructive ventilatory defect with severely limited DLCO. Chest x-ray (Figure 1) revealed a moderate-sized right pneumothorax with a pigtail chest tube in place and diffuse reticular interstitial opacities. His CT chest (figure 2) revealed extensive subcutaneous emphysema, diffuse lung cysts that are heterogenous in size, and subpleural reticular opacities with peripheral and basilar preponderance. Bronchoalveolar lavage revealed no infection, with predominant monocyte/ macrophages. Alpha-1 antitrypsin (A1AT) was normal, as were autoimmune panels. A hypersensitivity pneumonitis panel revealed positive IgG to Aureobasidium pullulans. A presumptive diagnosis of chronic hypersensitivity pneumonitis was made.

Spontaneous pneumothorax (SP), a potentially life-threatening complication, is defined by the accumulation of air in the pleural space with secondary lung collapse, and can be categorized as primary (without apparent lung disease) or secondary pneumothorax. While chronic obstructive pulmonary disease and Pneumocystitis jirovecii pneumonia are the most common causes of secondary spontaneous pneumothorax, other structural lung diseases such as fibrotic lung diseases have also been linked to SP. Interstitial lung diseases distort lung architecture and trigger formation of subpleural blebs that are susceptible to rupture leading to extra-alveolar air collection and air leakage in the pleural space. Presence of persistent air leak, as in our case, mandates surgical consideration to accelerate recovery and prevent recurrence of secondary SP.

Roula Altisheh MD and Tara Carr MD

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine

Banner-University Medical Center

Tucson, AZ USA

References

1. Sahn S, Heffiner J. Spontaneous Pneumothorax. N Engl J Med 2000; 342:868-74 [CrossRef] [PubMed]
2. Onuki T, Ueda S, Yamaoka M, Sek iya Y, Yamada H, Kawakami N, Araki Y, Wakai Y, Saito K, Inagaki M, Matsumiya N. Primary and secondary spontaneous pneumothorax: prevalence, clinical features, and in-hospital mortality. Can Respir J. 2017: 6014967.  [CrossRef] [PubMed]
3. Koschel D, Handzhiev S, Cardoso C, Rolle A, Holotiuk O, Höffken G. Pneumomediastinum as a primary manifestation of chronic hypersensitivity pneumonitis. Med Sci Monit. 2011 Dec;17(12):CS152-5. [PubMed]
4. Ichinose J, Nagayama K, Hino H, et al. Results of surgical treatment for secondary spontaneous pneumothorax according to underlying diseases. Eur J Cardiothorac Surg. 2016;49(4):1132–6. [CrossRef] [PubMed]

Cite as: Altisheh R, Carr T. Medical image of the week: spontaneous pneumothorax in end stage fibrotic lung disease. Southwest J Pulm Crit Care. 2017;14(6):308-10. doi: https://doi.org/10.13175/swjpcc065-17 PDF 

Medical Image of the Week: Lymphangitic Carcinomatosis

Figure 1. Mass like consolidation and interlobular septal thickening (arrows). 

A 64-year-old woman, never-smoker, was evaluated for shortness of breath and left leg swelling. An abnormal initial chest X-Ray lead to computed tomography (CT) scan of the chest. She was also diagnosed with deep vein thrombosis (DVT) of her left leg.

CT of the chest with intravenous contrast showed a mass-like consolidation in the right upper lobe and thickening of the peripheral interlobular septa and of the bronchovascular bundles consistent with lymphangitic carcinomatosis (Figure 1). Endobronchial ultrasound (EBUS) guided transbronchial needle aspirations of the station 10 R Lymph node were positive for adenocarcinoma of lung origin.

Lymphangitic carcinomatosis occurs when cancer cells spread along the pulmonary lymphatic system and result in thickening of the bronchovascular bundle, the interlobular septa, or both (1). Histopathologically, specimens show interlobular and subpleural interstitial desmoplastic thickening and obstruction of lymphatic vessels by tumor cells. It carries a poor prognosis.

Mohammad R. Dalabih, MBBS¹ and Joshua Malo, MD²

¹Pulmonary Consultants LLC, Tacoma, WA USA

²Division of Pulmonary, Allergy, Critical Care. And Sleep, University of Arizona College of Medicine, Tucson, AZ USA

Reference

1. Munk PL, Müller NL, Miller RR, Ostrow DN. Pulmonary lymphangitic carcinomatosis: CT and pathologic findings. Radiology. 1988 Mar;166(3):705-9. [CrossRef] [PubMed]

Cite as: Dalabih MR, Malo J. Medical image of the week: lymphangitic cacinomatosis. Southwest J Pulm Crit Care. 2017;14(5):240. doi: https://doi.org/10.13175/swjpcc053-17 PDF

Medical Image of the Week: Bronchopulmonary Sequestration

Figure 1. A) Chest x-ray PA view demonstrating an oblong soft tissue density in the retrocardiac region overlying the medical aspect of the left hemidiaphragm. B) Chest x-ray lateral view demonstrating the same opacity anterior to a lower thoracic vertebral body, suspicious for a lung mass.

Figure 2. Chest computed tomography A) axial and B) coronal sections demonstrating a lobulated, cystic structure within the left lower lobe with a feeding artery off the aorta, consistent with a pulmonary sequestration.

A 49-year-old woman was incidentally found to have a lung mass on a pre-operative chest x-ray done prior to an elective cholecystectomy (Figure 1). Chest computed tomography, ordered to further characterize this mass revealed a left lower lobe lobulated, cystic opacity with a feeding artery from the aorta, consistent with bronchopulmonary sequestration (BPS) (Figure 2). Given that she has not had any complications of BPS we elected to manage her conservatively with observation.

BPS is a rare congenital malformation of the lower airways characterized by abnormal lung tissue that does not communicate with the tracheobronchial tree and receives its blood supply from the systemic circulation (1). Our patient’s BPS was intralobar in location, occurring within a normal lobe but lacking its own visceral pleura. The posterior basal left lower lobe is the most common intralobar location. Among cases that escape clinical detection in infancy, BPS comes to light in childhood or adulthood as either an incidental radiographic finding or as a symptomatic presentation of a lung infection. While surgical resection is generally considered to be the treatment of choice given the risk of developing infection, hemorrhage or malignancy (2), some asymptomatic adults with BPS may be managed conservatively with observation with serial imaging (3).

Udit Chaddha MD¹, Niusha Damaghi MD¹, Ashley Prosper MD², and Ching-Fei Chang MD¹

¹Division of Pulmonary, Critical Care and Sleep Medicine and ²Department of Radiology

Keck School of Medicine

University of Southern California

Los Angeles, CA USA

References

1. Biyyam DR, Chapman T, Ferguson MR, Deutsch G, Dighe MK. Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 2010 Oct;30(6):1721-38. [CrossRef] [PubMed]
2. Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int. 2008 Jun;24(6):643-57. [CrossRef] [PubMed]
3. Stanton M, Njere I, Ade-Ajayi N, Patel S, Davenport M. Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg. 2009 May;44(5):1027-33. [CrossRef] [PubMed]

Cite as: Chaddha U, Damaghi N, Prosper A, Cha C-F. Medical image of the week: bronchopulmonary sequestration. Southwest J Pulm Crit Care. 2017;14(4):168-9. doi: https://doi.org/10.13175/swjpcc036-17 PDF

Medical Image of the Week: Papillomatosis

Figure 1. Chest roentgenogram.

Figure 2. Contrast enhanced computer tomography of chest.

A 24-year-old man with recurrent respiratory papillomatosis presented with a history of breathlessness and a change in voice for the last four months. He had undergone endoscopic debridement in the past for laryngeal papillomatosis. On initial evaluation, respiratory distress was thought to be due to recurrence of laryngeal papillomatosis as he improved after surgical de-bulking of laryngo-tracheal papillomas. However, he had some trickle of blood into bronchi with debridement under general anaesthesia. Post-operative chest roentgenogram showed bilateral patchy opacities giving the appearance of aspiration pneumonitis as shown in figure 1.

He also underwent contrast enhanced computer tomography of the chest which showed numerous but small cavitary lesions involving bilateral lung parenchyma as shown in figure 2. This lead to the diagnosis of pulmonary spread of laryngeal papillomatosis and the patient was given adjuvant treatment for this aggressive disease (1,2).

K Devaraja, MS, DNB and Kapil Sikka, MS, DNB

All India Institute of Medical Sciences

Ansari Nagar, New Delhi, India

References

1. Abe K, Tanaka Y, Takahashi M, Kosuda S, et al. Pulmonary spread of laryngeal papillomatosis: radiological findings. Radiat Med. 2006 May;24(4):297–301. [CrossRef] [PubMed]
2. Carifi M, Napolitano D, Morandi M, Dall'Olio D. Recurrent respiratory papillomatosis: current and future perspectives. Ther Clin Risk Manag. 2015;11:731–8. [CrossRef] [PubMed] 

Cite as: Devaraja K, Sikka K. Medical image of the week: papillomatosis. Southwest J Pulm Crit Care. 2017;14(3):123-4. doi: https://doi.org/10.13175/swjpcc025-17 PDF

Medical Image of the Week: Evolution of Low Grade Adenocarcinoma

Figure 1. Coned down chest CT images. Panels a-d: small ground glass focus in the right upper lobe demonstrating slow growth over a period of 10 years (yellow arrows) and gradual development of a soft tissue component (red arrows).

Ground glass lesions above 5 mm in greatest diameter found on chest computed tomography (CT) require initial followed up in 3 months according to the Fleischner Society Guidelines, to exclude a transient inflammatory focus (1). If persistent, surveillance for at least 24 months to confirm stability is recommended. Any change in size or density should warrant further action, ideally surgical consultation, given the suboptimal yield of percutaneous biopsy and risk of inappropriate staging if the whole lesion is not examined. This may result in the inability to recognize the transition from in-situ adenocarcinoma into minimally invasive or invasive lesions, which in turn results in inaccurate staging and prognosis.

Diana Palacio MD, Berndt Schmit MD, and Veronica Arteaga MD

Department of Medical Imaging

Banner-University Medical Center Tucson

Tucson, AZ USA

Reference

1. MacMahon H, Austin JH, Gamsu G, Herold CJ, Jett JR, Naidich DP, Patz EF Jr, Swensen SJ; Fleischner Society. Guidelines for management of small pulmonary nodules detected on CT scans: a statement from the Fleischner Society. Radiology. 2005 Nov;237(2):395-400. [CrossRef] [PubMed]

Cite as: Palacio D, Schmit B, Arteaga V. Medical image of the week: evolution of low grade adenocarcinoma. Southwest J Pulm Crit Care. 2017;14(3):103. doi: https://doi.org/10.13175/swjpcc026-17 PDF 

Medical Image of the Week: Subcutaneous Calcification in Dermatomyositis

Figure 1. Thoracic CT scan in lung windows showing non-specific interstitial disease secondary to dermatomyositis.

Figure 2. Pelvic CT scan showing subcutaneous calcifications (encircled).

A 36-year old woman was referred to our Interstitial Lung Disease (ILD) clinic for evaluation of dyspnea. A high-resolution CT scan of the chest showed perivascular reticular and ground glass opacities with air trapping, consistent with non-specific interstitial pneumonitis (Figure 1). She was diagnosed with connective tissue associated ILD. On review of previous images extensive subcutaneous calcifications were seen (Figure 2).

Calcinosis is an uncommon manifestation of dermatomyositis in adults (1). It is usually seen around areas of frequent trauma like the hands and elbows. In her case, a pelvic inflammatory disease may have been a trigger for this calcinosis. Calcinosis is a difficult complication to treat with some success seen with diltiazem, aluminum hydroxide, and even alendronate in children. Surgical excision may be required in some cases.

Bhupinder Natt MD

Division of Pulmonary, Allergy, Critical Care and Sleep

Banner-University Medical Center, Tucson (AZ)

Reference

1. Chander S, Gordon P. Soft tissue and subcutaneous calcification in connective tissue diseases. Curr Opin Rheumatol. 2012 Mar;24(2):158-64. [CrossRef] [PubMed]

Cite as: Natt B. Medical image of the week: subcutaneous calcification in dermatomyositis. Southwest J Pulm Crit Care. 2016;13(6):317-8. doi: https://doi.org/10.13175/swjpcc130-16 PDF 

December 2016 Imaging Case of the Month

Eric A. Jensen, MD

Michael B. Gotway, MD 

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Imaging Case of the Month CME Information  

Members of the Arizona, New Mexico, Colorado and California Thoracic Societies and the Mayo Clinic are able to receive  0.25 AMA PRA Category 1 Credits™. Completion of an evaluation form is required to receive credit and a link is provided on the last panel of the activity.

0.25 AMA PRA Category 1 Credit(s)™

Estimated time to complete this activity: 0.25 hours

Lead Author(s): Eric A. Jensen, MD. All Faculty, CME Planning Committee Members, and the CME Office Reviewers have disclosed that they do not have any relevant financial relationships with commercial interests that would constitute a conflict of interest concerning this CME activity. 

Learning Objectives:
As a result of this activity I will be better able to:    

1. Correctly interpret and identify clinical practices supported by the highest quality available evidence.
2. Will be better able to establsh the optimal evaluation leading to a correct diagnosis for patients with pulmonary, critical care and sleep disorders.
3. Will improve the translation of the most current clinical information into the delivery of high quality care for patients.
4. Will integrate new treatment options in discussing available treatment alternatives for patients with pulmonary, critical care and sleep related disorders.

Learning Format: Case-based, interactive online course, including mandatory assessment questions (number of questions varies by case). Please also read the Technical Requirements.

CME Sponsor: University of Arizona College of Medicine at the Arizona Health Sciences Center.

Current Approval Period: January 1, 2015-December 31, 2016

Clinical History: A 47-year-old woman presented for medical evaluation prior to trans-sphenoid hypophysectomy for pituitary adenoma for Cushing syndrome. The patient had an extensive past medical history, including kidney minimal change disease treated with corticosteroids between 5-7 years previously (no longer on corticosteroid therapy), type II diabetes mellitus, focal segmental glomeruloscleroosis on renal biopsy, morbid obesity, gout, obstructive sleep apnea on continuous positive airway pressure (CPAP) supplemented with oxygen for the previous 8 years, hypertension, and recent-onset atrial fibrillation, as well as a history of several pneumonias, perhaps related to chronic immunosuppression. Her past surgical history included bilateral partial knee replacement, lower extremity vein ablation, and breast reduction. Her medication list was extensive, including allopurinol, anti-hypertensives, anti-depressants, colchicine, oxygen, and Tacrolimus, among others, including over-the-counter medications.

Laboratory data, include white blood cell count, coagulation profile, and serum chemistries were within normal limits. Oxygen saturation on room air was 95%.

Frontal and lateral chest radiographs (Figure 1) were performed. A previous chest radiograph performed 2 years earlier is presented for comparison (Figure 2).

Figure 1. Frontal (A) and lateral (B) chest radiography.

Figure 2.  Frontal chest radiography performed 2 years prior to presentation.

Which of the following statements regarding the chest radiograph is most accurate? (Click on the correct answer to proceed to the second of seven pages)

1. Frontal and lateral chest radiography appears normal
2. Frontal and lateral chest radiography shows a mass projected over the right paratracheal region
3. Frontal and lateral chest radiography shows asymmetric hyperlucency affecting the right thorax
4. Frontal and lateral chest radiography shows basal reticulation suggesting possible fibrotic disease
5. Frontal and lateral chest radiography shows cardiomegaly only, but is unchanged from prior

Cite as: Jensen EA, Gotway MB. December 2016 imaging case of the month. Southwest J Pulm Crit Care. 2016;13(6):290-301. doi: https://doi.org/10.13175/swjpcc135-16 PDF 

Medical Image of the Week: Leukemic Infiltrates

Figure 1. AP portable chest x-ray demonstrating diffuse bilateral infiltrates.

Figure 2. Histology showing extensive interstitial and perivascular lymphocytic infiltrates.

Figure 3. Immunohistochemical staining for CD8 positive T-cell immunophenotype.

A 50 year-old white man with newly diagnosed, acute T-cell prolymphocytic leukemia presented with progressive exertional dyspnea and non-productive cough. The patient was due to meet with his hematologist that day to discuss initiation of treatment. The patient had not noted fever, chills, night sweats, chest pain, or lower extremity swelling. Blood pressure was 112/60 mm Hg, respiratory rate was 36/minute and labored, pulse was 110/minute and temperature was 37 degrees Celsius. Oxygen saturation measured by pulse oximetry was 62% on room air at rest, and rose to 90% after the application of a 100% non-rebreather mask. Diffuse rales were present on chest auscultation. Marked splenomegaly was present on abdominal examination. Peripheral white blood count was 112.2 K/ul with 99% lymphocytes. Smudge cells were noted. Hemoglobin was 12.9 g/dl and platelet count was 93K/ul. Procalcitonin level was 0.3 pg/ml. The chest radiograph demonstrated diffuse bilateral infiltrates (Figure 1). The patient developed rapidly progressive hypoxemia, was intubated orally, and mechanical ventilation was initiated. Lung biopsies were performed via a video-assisted thoracic surgical approach of the right middle and right lower lobes. Microscopic examination demonstrated extensive leukemic infiltration of the pulmonary interstitium and perivascular space (Figure 2). Immunohistochemical staining showed that the infiltrating cells expressed a CD8 positive T-cell immunophenotype (Figure 3) pattern similar to the patient’s peripheral blood flow cytometry study. Therapy began with an escalating dose of alemtuzumab and intermittent pentostatin, but the patient developed progressive multi-organ failure and expired.

Acute T-cell prolymphocytic leukemia is an aggressive mature T-cell leukemia usually characterized by peripheral blood lymphocytosis and splenomegaly (1). Extramedullary involvement most commonly affects the skin (2). Diffuse interstitial and perivascular pulmonary involvement with respiratory failure has not been previously reported. Pathological involvement of the pulmonary interstitial space should be considered in patients with acute T-cell prolymphocytic leukemia and respiratory insufficiency.

Charles J. VanHook1, Carlyne Cool2, Todd DeBoom3, Robert Fisher4, and

Douglas J. Tangel1

1Department of Intensive Care Medicine

Longmont United Hospital

Longmont, CO

2Department of Pathology and Department of Medicine

University of Colorado and National Jewish Health

Denver, CO

3Department of Pathology

Longmont United Hospital

Longmont, CO

4Department of Oncology

Longmont United Hospital

Longmont, CO

References

1. Dearden CE. T-cell prolymphocytic leukemia. Med Oncol. 2006;23(1):17-22. [CrossRef] [PubMed]
2. Valbuena JR, Herling M, Admirand JH, Padula A, Jones D, Medeiros LJ. T-cell prolymphocytic leukemia involving extramedullary sites. Am J Clin Pathol. 2005;123(3):456-64. [CrossRef] [PubMed]

Reference as: VanHook CJ, Cool C, DeBoom T, Fisher R, Tangel DJ. Medical image of the week: leukemic infiltrates. Southwest J Pulm Crit Care. 2015;10(5):235-7. doi: http://dx.doi.org/10.13175/swjpcc043-15 PDF