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Case Reports and Images in Pathology

 
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Case Report
 
Perivascular epithelioid cell tumor of the lung with focal pigment production
Sowmini Medavaram1, Trishala Agrawal2, Gail Prado3, Maya Shah4
1MD, Internal Medicine Resident, Newark Beth Israel Medical Center, Newark, NJ.
2MD, Hematology/Oncology fellow, Newark Beth Israel Medical Center, Newark, NJ.
3MD, Pathology Resident, Newark Beth Israel Medical Center, Newark, NJ.
4MD, Hematology/ Oncology Attending, Assistant Program Director of Fellowship, Newark Beth Israel Medical Center, Newark, NJ.

Article ID: 100007Z11SM2016
doi:10.5348/Z11-2016-7-CR-3

Address correspondence to:
Sowmini Medavaram
MD, 3111 Bristol Station Ct, Carteret
NJ-07008

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How to cite this article:
Medavaram S, Agrawal T, Prado G, Shah M. Perivascular epithelioid cell tumor of the lung with focal pigment production. J Case Rep Images Pathol 2016;2:10–14.


Abstract
Introduction: Introduction: Perivascular epithelioid cell (PEC) tumors, also (PEComas), are rare mesenchymal tumors defined by the presence of perivascular epithelioid cells that express muscle and melanocytic markers and have no known normal tissue counterparts. These include wide range of tumors like angiomyolipoma (AML), lymphangioleiomyomatosis (LAM) and clear cell "sugar" tumor of the lung (CCST). These are associated with tuberous sclerosis. The PEComa with pigment production is rare and it can be a diagnostic challenge to distinguish it from malignant melanoma. The immunoprofile of the tumor with strong human melanocytic black-45 (HMB-45) expression and the absence of other melanocytic markers like S-100 and SOX-10 suggests the diagnosis of PEComa.
Case Report: We report a 34-year-old female with past medical history of hypothyroidism who presented with right upper back pain for past six months. Chest X-ray performed at that time revealed a lung mass. Computed tomography scan of chest showed a multi-lobular 3.4x2.2 cm soft tissue mass in the right lower lobe. Bronchoscopy was non-diagnostic. Computed tomography guided biopsy revealed atypical spindle cells strongly positive for HMB-45 and vimentin, negative for melanoma markers like S100. She eventually underwent a VATS procedure with complete resection of the tumor. Pathology of the lung mass confirmed the same.
Conclusion: Pigmented PEComas are rare and difficult to distinguish from malignant melanoma. Findings of atypical spindle cell neoplasm with focal pigment production which is positive for HMB-45 and negative for S-100 stain makes PEComa more likely. Pigmented PEComas of the lung have not been reported so far to our knowledge.

Keywords: PEComa of lung, Pigmented PEComa, HMB-45, Perivascular epithelioid


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Author Contributions
Sowmini Medavaram – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Trishala Agrawal – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Gail Prado – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Maya Shah – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Sowmini Medavaram et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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