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 

Medical Image of the Week: Tracheobronchopathia Osteochondroplastica

Figure 1. View of trachea during bronchoscopy showing submucosal nodules.

Figure 2. H & E staining of cartilage biopsy.

A 52-year-old asymptomatic woman underwent a low dose computed tomography (CT) of chest due to long-standing history of smoking. CT chest revealed a 4 mm right lower lobe pulmonary nodule. Also noted were several nodules throughout the trachea and in the left main-stem bronchus. Bronchoscopy revealed multiple non-obstructing submucosal nodules along the tracheal rings with sparing of the posterior membranous portion of the trachea (Figure 1).  Endotracheal biopsy showed benign cartilage and ciliated epithelium (Figure 2). The patient was diagnosed with tracheobronchopathia osteochondroplastica (TO). Clinical manifestations of TO are nonspecific and include cough, wheezing, hemoptysis, dyspnea, and recurrent lung infections (1). Therapy for TO includes alleviation of symptoms with bronchodilators, treatment of respiratory infections and tracheal dilation.  Therapeutic modalities for tracheal dilation includes surgical resection, laser ablation and vaporization.  CT chest was to be repeated at 12 months for follow-up of the pulmonary nodule. The patient was lost to follow-up.

Benjamin O. Lawson MD¹, Kelechi Abarikwu², and Aditya Gupta MD³

¹Internal Medicine and ³Pulmonary/Critical Care Medicine

HonorHealth Scottsdale Thompson Peak Medical Center

Scottsdale, AZ USA

²University of Arizona Tucson

Tucson, AZ USA

Reference

1. Simmons C, Vinh D, Donovan DT, Ongkasuwan J. Tracheobronchopathia osteochondroplastica. Laryngoscope. 2016 Sep;126(9):2006-9. [CrossRef] [PubMed]

Cite as: Lawson BO, Abarikwu K, Gupta A. Medical image of the week: Tracheobronchopathia osteochondroplastica. Southwest J Pulm Crit Care. 2018;17(2):45-6. doi: https://doi.org/10.13175/swjpcc094-18 PDF

Medical Image of the Week: Pulmonary Amyloidosis in Primary Sjogren’s Syndrome

Figure 1. Thoracic CT scan showing multiple pulmonary nodules and lung cysts. The lung cysts were located apart from the pulmonary nodules.

Figure 2. Follow up CT scan in one year revealing worsening of the widespread lung cysts.

A 69-year-old woman with past medical history of Sjögren's syndrome presented with pleuritic chest pain and shortness of breath for a month. Review of systems revealed worsening dysphagia and dryness of eyes over the last one year. Physical exam was significant for a palpable left axillary node and mild rhonchi bilaterally in the lower lung bases. Laboratory work was positive for Sjogren’s Syndrome antibodies. Chest x-ray revealed multiple nodules in bilateral lung fields. HRCT showed interlobular septal thickening and multiple cystic areas throughout the lung parenchyma which had progressed over 1 year (Figure 1). Wedge resection and thorough lymph node dissection were performed via video-assisted thoracic surgery (VATS). Biopsy of the lung nodules revealed thickened alveolar septate with acellular eosinophilic homogenous materials, which took up Congo Red stain. Based on these pathological findings, the final diagnosis was - diffuse septal alveolar pulmonary amyloidosis secondary to Sjogren’s Syndrome. bortezomib. However, patient did not want to undergo chemotherapy. She preferred the ‘wait and watch approach’ and wished to be treated with only prednisone, with the intention to switch to azathioprine in future. However, after one year thoracic CT showed worsening of the cysts (Figure 2).

Pulmonary amyloidosis of the lower respiratory tract may represent a significant clinical problem in systemic and organ-limited amyloidosis and can contribute to cardiopulmonary failure. Pulmonary amyloidosis may present as a nodular localized type, diffuse septal alveolar amyloidosis, tracheobronchial amyloidosis and even pleural amyloidosis (1). Each patient requires complete assessment and unequivocal amyloid typing to determine their optimal treatment. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis, with most chemotherapeutic regimes similar to that of multiple myeloma (2). Patients should be monitored very closely and physicians should frequently asses the efficacy of the chemotherapeutic regime.

Payal Sen, MD and Betty Chang, MDCM, PhD

University of New Mexico

Albuquerque, NM USA

References

1. Milani P, Basset M, Russo F, Foli A, Palladini G, Merlini G. The lung in amyloidosis. Eur Respir Rev. 2017 Sep 6;26(145). [CrossRef] [PubMed]
2. Dispenzieri A, Seenithamby K, Lacy MQ, et al. Patients with immunoglobulin light chain amyloidosis undergoing autologous stem cell transplantation have superior outcomes compared with patients with multiple myeloma: a retrospective review from a tertiary referral center. Bone Marrow Transplant. 2013 Oct;48(10):1302-7. [CrossRef] [PubMed]

Cite as: Sen P, Chang B. Medical image of the week: Pulmonary amyloidosis in primary Sjogren’s syndrome. Southwest J Pulm Crit Care. 2018;16(6):336-7. doi: https://doi.org/10.13175/swjpcc072-18 PDF 

Medical Image of the Week: Erythema Nodosum

Figure 1. Panel A: photograph of legs at initial presentation. Panel B: 2 weeks later.

A healthy 43-year-old woman presented to the emergency room with one day of diarrhea, vomiting and severe left-sided pleuritic chest pain. Chest radiography revealed an infiltrate in the left lower lobe with a small pleural effusion. White count was 14,000 cells/mcL. Eosinophil count was 2%. She was thought to have pneumonia and treated with azithromycin.

Two weeks later she returned to the emergency room with painful raised erythematous lesions on both lower extremities and generalized joint pain. A thoracic CT scan was performed showing left lower lobe pneumonia and small bilateral pleural effusions. Eosinophilia was 14%. She was diagnosed again as having pneumonia. There was no diagnosis made of the skin lesions. A cocci serology was drawn and sent to Davis California. She was given doxycycline.

Subsequently the cocci serology was found to be positive for IgG and IgM and negative for complement fixation antibodies. She was seen by her primary care physician who diagnosed acute coccidioidomycosis and started her on Diflucan 200 mg daily and referred her for consultation. At her first visit 6 weeks into her illness she was still complaining of arthralgias, fatigue and cough.

Physical examination was negative except for innumerable red raised lesions on her thighs and anterior surfaces of her legs with confluence at the ankles (Figure 1). One month after her initial visit her legs were much improved although there was some peeling of the skin and residual erythema of the lower extremities (Figure 2).

Erythema Nodosum (EN) is a panniculitis of subcutaneous fat which can be associated with a variety of conditions including streptococcal pharyngitis, tuberculosis, sarcoidosis, inflammatory bowel disease, cancer, or bacterial infections (1). The usual presentation of this disease is the presence of painful raised erythematous nodules symmetrically on the anterior surfaces of the lower extremities. These lesions do not represent sites of infection but are most likely a result of type IV delayed hypersensitivity.

EN was first described as a benign form of coccidioidomycosis in 1936, and was further characterized by Charles Smith in 1940 when he described acute coccidioidomycosis as an illness characterized by an “influenza like initial phase followed in 2 to 18 days by the eruptive phase of erythema nodosum lasting from six days to three weeks with pigmented areas lasting for months” (2). He noted that recovery was invariable. This was in a time where the frequent presentation of acute coccidioidomycosis as an inapparent infection was not known and the mortality of acute cocci was as high as 50%. Twenty years later, Smith and Pappagiannis made the observation that EN was 2 to 10 times more frequent in females than males (3). Braverman (4) in 1999 observed the protective effect of EN by reporting on 60 pregnant women with coccidioidomycosis. Thirty of these women with EN had no dissemination, and of the 30 without EN, 11 disseminated and one died. The mechanism of this protective effect has yet to be characterized.

Physicians living in the Southwest have learned that “the bumps” (EN), desert rheumatism (polyarthralgia), eosinophilia associated with a flulike illness, and acute knifelike pleuritic chest pain in an otherwise healthy person are all signs and symptoms which lead to a rapid diagnosis of coccidioidomycosis (5). Because of the time sequence of the appearance of EN in this illness this rash is often felt incorrectly to be an allergic reaction to antibiotics given for the previously diagnosed pneumonia. Even though EN is associated with many varied conditions, its presence in the Southwestern United States should lead the physician to consider coccidioidomycosis as the most likely diagnosis.

Gerald F. Schwartzberg, MD

HonorHealth Pulmonology

Phoenix, AZ USA

References

1. Blake T, Manahan M, Rodins K. Erythema nodosum - a review of an uncommon panniculitis. Dermatol Online J. 2014 Apr 16;20(4):22376. [PubMed]
2. Smith CE. Epidemiology of acute coccidioidomycosis with erythema nodosum ("San Joaquin" or "Valley Fever"). Am J Public Health Nations Health. 1940 Jun;30(6):600-11. [CrossRef] [PubMed]
3. Smith CE, Pappagianis D, Levine HB, Saito M. Human coccidioidomycosis. Bacteriol Rev. 1961 Sep;25:310-20. [PubMed]
4. Braverman IM. Protective effects of erythema nodosum in coccidioidomycosis. Lancet. 1999 Jan 16;353(9148):168. [CrossRef] [PubMed]
5. Stevens DA. Coccidioidomycosis. N Engl J Med. 1995 Apr 20;332(16):1077-82. [CrossRef] [PubMed]

Addendum: Another Erythema Nodosum

Figure 2. Another case of erythema nodosum.

While the above article was “in press”, another case of EN came into the office (Figure 2). Her history was similar to the first patient. Uncle Jun, the patriarch in the “Sopranos,” said in his heavy New York accent, “They come in threes.” Based on Uncle Jun, I am anticipating seeing another case of EN this week.

Gerald F. Schwartzberg, MD

HonorHealth Pulmonology

Phoenix, AZ USA

Cite as: Schwartzberg GF. Medical image of the week: erythema nodosum. Southwest J Pulm Crit Care. 2017;15(4):188-90. doi: https://doi.org/10.13175/swjpcc126-17 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

October 2016 Imaging Case of the Month

Michael B. Gotway, MD  

Department of Radiology

Mayo Clinic Arizona

Scottsdale, Arizona 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): 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 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 35-year-old woman presented with a several month history of slowly worsening shortness of breath and dry cough. Laboratory data, include white blood cell count and serum chemistries were within normal limits. Oxygen saturation on room air was 99%.

Frontal and lateral chest radiographs (Figure 1) were performed.

Figure 1. Frontal (A) and lateral (B) radiographs.

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

1. Frontal and lateral chest radiography appears normal
2. Frontal and lateral chest radiography shows abnormally diminished lung volumes
3. Frontal and lateral chest radiography shows bilateral peribronchial and mediastinal lymph node enlargement
4. Frontal and lateral chest radiography shows cardiomegaly
5. Frontal and lateral chest radiography shows upper lobe bilateral linear and reticular abnormalities

Cite as: Gotway MB. October 2016 imaging case of the month. Southwest J Pulm Crit Care. 2016;13(4): . doi: http://dx.doi.org/10.13175/swjpcc100-16 PDF

Medical Image of the Week: Tracheobronchopathia Osteochondroplastica

Figure 1: Panels (A and B) show the lumpy, bumpy nodules of tracheobronchopathia osteochondroplastica affecting the anterior tracheal wall with sparing of the posterior membrane. In this patient, copious amounts of white secretions can be seen in the distal trachea and the posterior membrane from her current MRSA pneumonia. 

Tracheobronchopathia osteochondroplastica (TO) is a rare, idiopathic tracheobronchial abnormality that is seen during 0.7% of bronchscopies. It is usually diagnosed in the 5th to 6th decades of life with a male preponderance (1,2)^. Here, we present the case of a 62-year-old woman with history of bronchial asthma with recurrent exacerbations who was admitted with pneumonia and a new mass-like consolidation on imaging. She underwent bronchoscopy for further work up and was found to have methicillin-resistant Staphylococcus aureus (MRSA) pneumonia. Incidental nodules were found in her trachea during the bronchoscopy (Figure 1). Most patients with TO are asymptomatic but can rarely present with cough, shortness of breath, and even non-massive hemoptysis due to ulceration of nodular mucosa. Secondary airway narrowing has also been reported. The lumpy, bumpy nodules typically are 3-8 mm in size, localize in the sub-mucosa of the trachea, and are difficult to biopsy due to their cartilaginous or osseous nature. Diagnosis can be made by chest CT or bronchoscopy. A very important distinctive feature is sparing of the posterior membranous wall of the trachea, differentiating it from other nodular airway diseases. TO is a benign disease that generally doesn’t need any specific treatment or intervention (1,2).

Huthayfa Ateeli, MBBS, Elaine Cristan, MD, and Afshin Sam, MD.

Department of Medicine, Division of Pulmonary, Critical Care, Sleep and Allergy Medicine

University of Arizona, Tucson, AZ USA

References

1. Lundgren R, Stjernberg NL. Tracheobronchopathia osteochondroplastica. A clinical bronchoscopic and spirometric study. Chest. 1981 Dec;80(6):706-9. [CrossRef] [PubMed]
2. Prince JS, Duhamel DR, Levin DL, Harrell JH, Friedman PJ. Nonneoplastic lesions of the tracheobronchial wall: radiologic findings with bronchoscopic correlation. Radiographics. 2002 Oct;22 Spec No:S215-30. [CrossRef] [PubMed] 

Cite as: Ateeli H, Cristan E, Sam A. Medical image of the week: tracheobronchopathia osteochondroplastica. Southwest J Pulm Crit Care. 2016;13(3):131-2. doi: http://dx.doi.org/10.13175/swjpcc067-16 PDF