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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2014; 35(01): 99-102
DOI: 10.4103/0971-5851.133733
CASE REPORT

Metastatic perivascular epithelioid cell tumor responding to mammalian target of rapamycin inhibition

Indranil Ghosh
Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India
,
Indu Arun
Department of Pathology, Tata Medical Center, Kolkata, West Bengal, India
,
Saugata Sen
Department of Radiodiagnosis, Tata Medical Center, Kolkata, West Bengal, India
,
Lopamudra Mishra
Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India
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Abstract

Perivascular epithelioid cell tumors (PEComa) are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Female genital tract and retroperitoneum are common sites of origin of PEComa-not otherwise specified. Diagnosis depends upon characteristic morphology and immunohistochemistry findings. Prognosis of unresectable or metastatic disease is poor. Responses to mammalian target of rapamycin (mTOR) inhibition are encouraging but mostly short-lived. We report a case of metastatic PEComa who responded to mTOR inhibition, albeit for a short duration. We also review the existing literature on mTOR inhibitors in PEComa.

Keywords

Immunohistochemistry - mammalian target of rapamycin inhibitors - perivascular epithelioid cell tumors - temsirolimus


Publication History

Article published online:
19 July 2021

© 2014. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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  • References

  • 1 Folpe AL. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: Fletcher CD, Unni KK, Mertens F, editors. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. Lyon: IARC Press; 2002. p. 221-2.
  • 2 Bleeker JS, Quevedo JF, Folpe AL. "Malignant" perivascular epithelioid cell neoplasm: Risk stratification and treatment strategies. Sarcoma 2012;2012:541626.
  • 3 Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: A clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol 2005;29:1558-75.
  • 4 Wagner AJ, Malinowska-Kolodziej I, Morgan JA, Qin W, Fletcher CD, Vena N, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: Targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 2010;28:835-40.
  • 5 Subbiah V, Trent JC, Kurzrock R. Resistance to mammalian target of rapamycin inhibitor therapy in perivascular epithelioid cell tumors. J Clin Oncol 2010;28:e415.
  • 6 Italiano A, Delcambre C, Hostein I, Cazeau AL, Marty M, Avril A, et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol 2010;21:1135-7.
  • 7 Ross C, Sharma S, Louca O, Scurr M, Hayes A, Judson I. A patient presenting with a perivascular epithelioid cell tumor in the broad ligament: A case report. J Med Case Rep 2011;5:383.
  • 8 Quek R, Wang Q, Morgan JA, Shapiro GI, Butrynski JE, Ramaiya N, et al. Combination mTOR and IGF-1R inhibition: Phase I trial of everolimus and figitumumab in patients with advanced sarcomas and other solid tumors. Clin Cancer Res 2011;17:871-9.
  • 9 Gennatas C, Michalaki V, Kairi PV, Kondi-Paphiti A, Voros D. Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. World J Surg Oncol 2012;10:181.
  • 10 Pedersen JV, Benson C, Tunariu N, Mitchell S, Fisher C, Thway K, et al. A retrospective study from the Royal Marsden Hospital (RMH) of patients with malignant perivascular epithelioid cell tumors (PEComa) receiving treatment with sirolimus (SI) or temsirolimus (TSI). J Clin Oncol 2012;30 Suppl: Abstr 10038.
  • 11 Dickson MA, Schwartz GK, Antonescu CR, Kwiatkowski DJ, Malinowska IA. Extrarenal perivascular epithelioid cell tumors (PEComas) respond to mTOR inhibition: Clinical and molecular correlates. Int J Cancer 2013;132:1711-7.
  • 12 Scheppach W, Reissmann N, Sprinz T, Schippers E, Schoettker B, Mueller JG. PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide. World J Gastroenterol 2013;19:1657-60.