Medical Image of the Week: Recurrent Sarcoidosis Resembling Malignancy

Figure 1. CT scan showing multiple splenic masses.

The patient is a 64-year-old, non-smoking, woman who presented with a dry cough for a year and had a history of sarcoidosis. Five years ago, she presented with cough, bilateral pulmonary nodules and mediastinal adenopathy. At that time by a fine needle aspiration (FNA) under endobronchial ultrasound biopsy (EBUS), revealing non caseating granulomatous inflammation. She had complete remission after she was treated with a course of steroids. Follow up chest CT showed remission of her disease. On this presentation, she was found to have incidental multiple splenic masses on a CT scan of the chest (Figure 1). Physical examination was normal, CBC and chemistry were normal, and she had negative tests for tuberculosis, rheumatologic disease, systemic sclerosis, and mixed connective tissue disease. 

Pulmonary function tests were consistent with restrictive impairment and mild decrease in diffusion capacity for carbon monoxide. Positron Emission Tomography (PET scan) showed increased uptake corresponding to these lesions with no other areas of abnormal uptake in the body. CT-guided biopsy of splenic lesion showed non-caseating granulomas consistent with sarcoidosis. She was retreated with a course of steroids and a follow up CT scan of the abdomen showed resolution of the splenic masses.

Sarcoidosis is a systemic inflammatory disorder of unknown etiology characterized by non-caseating granulomas. It is thought to result from an exaggerated host cellular immune response to an unknown antigen. Although the lungs are involved in 90 % of patients, other organs can be affected with the lungs or in isolation. Visceral involvement is usually asymptomatic and presents as hepatosplenomegaly on physical exam, or less often as isolated nodular splenic lesions suspicious of lymphoma. Rarely patients can have elevated liver enzymes from liver involvement or cytopenias from splenic enlargement (1).

Other differential diagnoses include infectious, neoplastic or benign conditions that may have similar morphology, such as tuberculosis, histoplasmosis, and lymphomas, hemangiomas and hamartomas. Diagnosis is usually confirmed by a biopsy and the disease responds to steroid treatment in over 70% of patients (2). Splenectomy is a therapeutic option in cases of steroids failure, severe hypersplenism, inability to exclude malignancy, or if risk of splenic rupture is high (3).

Isolated splenic sarcoidosis is a rare condition that can be the first presentation of sarcoidosis or a site of disease recurrence. It can mimic a variety of infectious, benign or malignant conditions especially when it presents with cytopenias due to hypersplenism, making biopsy necessary. Fortunately, splenic sarcoidosis responds to steroid treatment in most patients. Splenectomy can be considered in refractory cases.

Fuad Zeid MD¹, Hasan S. Yamin MD¹, Ahmed Amro MD², Fadi Alkhankan MD³, and Hani Alkhankan MD⁴

¹Pulmonary and Critical Care and ²Internal Medicine

Marshall University

Huntington, WV USA

³Pulmonary and Critical Care

Mercy hospital/ St. Louis University

St. Louis, MO USA

⁴McLaren Oakland Pulmonary/Critical Care,

Pontiac, MI USA

References

1. Vakil A, MD, Upadhyay H, Sherani K, Cervellione K, Fein A. A case of splenic sarcoidosis: initial diagnosis and one-year symptom follow-up. Chest. 2014;145(3):210A [Abstract].
2. Calik M, Aygun M, Yesildag M, et al. Nodular splenic sarcoidosis: a rare case report and review of the literature. Chest. 2014;146(4):399A [Abstract].
3. Sharma OP, Vucinic V, James DG. Splenectomy in sarcoidosis: indications, complications, and long-term follow-up. Sarcoidosis Vasc Diffuse Lung Dis. 2002 Mar;19(1):66-70. [PubMed]

Cite as: Zeid F, Yamin HS, Amro A, Alkhankan F, Alkhankan H. Medical image of the week: recurrent sarcoidosis resembling malignancy. Southwest J Pulm Crit Care. 2018;18(5):279-80. doi: https://doi.org/10.13175/swjpcc057-18 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