Spontaneous Pneumothoraces due to Metastatic Endometrial Stromal Sarcoma in a Woman Infected with HIV.

A 41-year-old woman with HIV/AIDS (CD4 439 cells/mm3 on antiretroviral therapy) presented with cough, dyspnea, and pleuritic chest pain. Chest imaging showed bilateral pneumothoraces as well as numerous solid and cystic subpleural pulmonary nodules and masses (Figure 1). Concurrent abdominopelvic imaging revealed uterine and bilateral adnexal enlargement. CA-125 was 41.5 U/ml (normal, <20 U/ml). Pneumothoraces were managed with bilateral tube thoracostomy. Surgical lung biopsy yielded a diagnosis of metastatic endometrial stromal sarcoma (ESS) (Figure 2A). Two years earlier, a uterine mass had been diagnosed as leiomyoma by biopsy. In advanced HIV infection (CD4 <200 cells/mm3), pneumothoraces are typically associated with opportunistic infections. Our patient’s reconstituted immunity, previous uterine pathology, and elevated CA-125 prompted consideration of alternative etiologies such as pulmonary metastases or thoracic endometriosis. Obtaining a definitive diagnosis of ESS is challenging. Common obstacles include inadequate sampling and confusion with other spindle cell entities due to overlapping cytopathologic profiles. In one retrospective series, 40% of uterine ESS cases were initially misdiagnosed as benign lesions, resulting in a mean diagnostic delay of 143 months (1). In our case, positive CD10 staining of lung biopsy tissue helped secure the diagnosis of metastatic ESS (2). Early detection is critical, because 5-year survival for stage III/IV disease is only 66% (3, 4). Our patient was initially managed with hysterectomy and megestrol acetate (5). Unfortunately, her disease progressed, and she was switched to exemestane, an aromatase inhibitor. Footnotes Author Contributions: D.J.S., conception/design/drafting/finalizing; V.K.P., conception/design/drafting/finalizing; G.O.-I., drafting/finalizing; E.A.B., drafting/finalizing; M.-L.T.N., drafting/finalizing; A.N.S., conception/design/drafting/finalizing. Some of the contents of this case report have been previously reported in the form of an abstract/poster presentation (5). Originally Published in Press as DOI: 10.1164/rccm.201505-0892IM on September 23, 2015 Author disclosures are available with the text of this article at www.atsjournals.org. References - 1.Amant F, Moerman P, Cadron I, Neven P, Berteloot P, Vergote I. The diagnostic problem of endometrial stromal sarcoma: report on six cases. Gynecol Oncol. 2003;90:37–43. doi: 10.1016/s0090-8258(03)00207-5. [DOI] [PubMed] [Google Scholar] - 2.Sumathi VP, McCluggage WG. CD10 is useful in demonstrating endometrial stroma at ectopic sites and in confirming a diagnosis of endometriosis. J Clin Pathol. 2002;55:391–392. doi: 10.1136/jcp.55.5.391. [DOI] [PMC free article] [PubMed] [Google Scholar] - 3.Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR. Primary uterine endometrial stromal neoplasms: a clinicopathologic study of 117 cases. Am J Surg Pathol. 1990;14:415–438. doi: 10.1097/00000478-199005000-00002. [DOI] [PubMed] [Google Scholar] - 4.Rauh-Hain JA, del Carmen MG. Endometrial stromal sarcoma: a systematic review. Obstet Gynecol. 2013;122:676–683. doi: 10.1097/AOG.0b013e3182a189ac. [DOI] [PubMed] [Google Scholar] - 5.Shah DJ, Memon A, Phadke VK, Nguyen ML, Sheth AN. Biopsy, biopsy, and biopsy again: an unexpected cause of spontaneous pneumothorax in an HIV-infected woman [abstract]. Presented at the 38th Annual Meeting of the Society of General Internal Medicine, Toronto, ON, Canada, 2015. J Gen Intern Med. 2015;30(Suppl 2):368–369. [Google Scholar]