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Erschienen in: Skeletal Radiology 11/2012

01.11.2012 | Case Report

Primary perivascular epithelioid cell neoplasm (PEComa) of bone: Report of two cases and review of the literature

verfasst von: Nicholas M. Desy, Mitchell Bernstein, Ayoub Nahal, Mohamed Aziz, Samuel Kenan, Robert E. Turcotte, Leonard B. Kahn

Erschienen in: Skeletal Radiology | Ausgabe 11/2012

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Abstract

Primary perivascular epithelioid cell neoplasms (PEComas) of bone are rare mesenchymal tumors. Histologically, they are composed predominantly of perivascular epithelioid cells and have the capacity to metastasize. PEComas have been reported within intra-abdominal and intra-pelvic organs. To the best of our knowledge, only seven primary PEComas of bone have been described in the English literature. We present two cases of PEComa of bone, one arising from the distal fibula and one from the acetabulum. Both were treated by surgical excision and one also received adjuvant chemotherapy.
Literatur
1.
2.
Zurück zum Zitat Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41:1–15.PubMedCrossRef Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41:1–15.PubMedCrossRef
3.
Zurück zum Zitat 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.PubMedCrossRef 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.PubMedCrossRef
4.
Zurück zum Zitat Koch MB, Shih IM, Weiss SW, Folpe AL. Microphthalmia transcription factor and melanoma cell adhesion molecule expression distinguish desmoplastic/spindle cell melanoma from morphologic mimics. Am J Surg Pathol. 2001;25:58–64.PubMedCrossRef Koch MB, Shih IM, Weiss SW, Folpe AL. Microphthalmia transcription factor and melanoma cell adhesion molecule expression distinguish desmoplastic/spindle cell melanoma from morphologic mimics. Am J Surg Pathol. 2001;25:58–64.PubMedCrossRef
5.
Zurück zum Zitat Argani P, Aulmann S, Illei PB, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol. 2010;34:1395–406.PubMedCrossRef Argani P, Aulmann S, Illei PB, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol. 2010;34:1395–406.PubMedCrossRef
6.
Zurück zum Zitat Cho HY, Chung DH, Khurana H, Zhai QJ, Ro JY. The role of TFE3 in PEComa. Histopathol. 2008;53:236–49.CrossRef Cho HY, Chung DH, Khurana H, Zhai QJ, Ro JY. The role of TFE3 in PEComa. Histopathol. 2008;53:236–49.CrossRef
7.
Zurück zum Zitat Yamashita K, Fletcher CD. PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Am J Surg Pathol. 2010;34:1622–9.PubMed Yamashita K, Fletcher CD. PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review. Am J Surg Pathol. 2010;34:1622–9.PubMed
8.
Zurück zum Zitat Birkhaeuser F, Ackermann C, Flueckiger T, et al. First description of a PEComa (perivascular epithelioid cell tumor) of the colon: report of a case and review of the literature. Dis Colon Rectum. 2004;47:1734–7.PubMedCrossRef Birkhaeuser F, Ackermann C, Flueckiger T, et al. First description of a PEComa (perivascular epithelioid cell tumor) of the colon: report of a case and review of the literature. Dis Colon Rectum. 2004;47:1734–7.PubMedCrossRef
9.
Zurück zum Zitat Fink D, Marsden DE, Edwards L, Camaris C, Hacker NF. Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. Int J Gynecol Cancer. 2004;14:1036–9.PubMedCrossRef Fink D, Marsden DE, Edwards L, Camaris C, Hacker NF. Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. Int J Gynecol Cancer. 2004;14:1036–9.PubMedCrossRef
10.
Zurück zum Zitat Gao Z, Bhuiya T, Anderson A. Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract. J Obstet Gynaecol. 2004;24:600–4.PubMedCrossRef Gao Z, Bhuiya T, Anderson A. Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract. J Obstet Gynaecol. 2004;24:600–4.PubMedCrossRef
11.
Zurück zum Zitat Nese N, Martignoni G, Fletcher CD, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35:161–76.PubMedCrossRef Nese N, Martignoni G, Fletcher CD, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35:161–76.PubMedCrossRef
12.
Zurück zum Zitat Parfitt JR, Bella AJ, Wehrli BM, Izawa JI. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report. BMC Urol. 2006;6:20.PubMedCrossRef Parfitt JR, Bella AJ, Wehrli BM, Izawa JI. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report. BMC Urol. 2006;6:20.PubMedCrossRef
13.
Zurück zum Zitat Tsui WM, Colombari R, Portmann BC, et al. Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants. Am J Surg Pathol. 1999;23:34–48.PubMedCrossRef Tsui WM, Colombari R, Portmann BC, et al. Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants. Am J Surg Pathol. 1999;23:34–48.PubMedCrossRef
14.
Zurück zum Zitat Zamboni G, Pea M, Martignoni G, et al. Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20:722–30.PubMedCrossRef Zamboni G, Pea M, Martignoni G, et al. Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20:722–30.PubMedCrossRef
15.
Zurück zum Zitat Bandhlish A, Leon Barnes E, Rabban JT, McHugh JB. Perivascular epithelioid cell tumors (PEComas) of the head and neck: report of three cases and review of the literature. Head Neck Pathol. 2011;5:233–40.PubMedCrossRef Bandhlish A, Leon Barnes E, Rabban JT, McHugh JB. Perivascular epithelioid cell tumors (PEComas) of the head and neck: report of three cases and review of the literature. Head Neck Pathol. 2011;5:233–40.PubMedCrossRef
16.
Zurück zum Zitat Folpe AL, McKenney JK, Li Z, Smith SJ, Weiss SW. Clear cell myomelanocytic tumor of the thigh: report of a unique case. Am J Surg Pathol. 2002;26:809–12.PubMedCrossRef Folpe AL, McKenney JK, Li Z, Smith SJ, Weiss SW. Clear cell myomelanocytic tumor of the thigh: report of a unique case. Am J Surg Pathol. 2002;26:809–12.PubMedCrossRef
17.
Zurück zum Zitat Ghazali N, Cascarini L, Norris P, Barrett AW, Lavery KM. Perivascular epithelioid cell tumor (PEComa) of the cheek. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010;110:e26–31.PubMed Ghazali N, Cascarini L, Norris P, Barrett AW, Lavery KM. Perivascular epithelioid cell tumor (PEComa) of the cheek. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010;110:e26–31.PubMed
18.
Zurück zum Zitat Insabato L, De Rosa G, Terracciano LM, Fazioli F, Di Santo F, Rosai J. Primary monotypic epithelioid angiomyolipoma of bone. Histopathol. 2002;40:286–90.CrossRef Insabato L, De Rosa G, Terracciano LM, Fazioli F, Di Santo F, Rosai J. Primary monotypic epithelioid angiomyolipoma of bone. Histopathol. 2002;40:286–90.CrossRef
19.
Zurück zum Zitat Lian DW, Chuah KL, Cheng MH, Yap WM. Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour. J Clin Pathol. 2008;61:1127–9.PubMedCrossRef Lian DW, Chuah KL, Cheng MH, Yap WM. Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour. J Clin Pathol. 2008;61:1127–9.PubMedCrossRef
20.
Zurück zum Zitat Righi A, Dimosthenous K, Rosai J. PEComa: another member of the MiT tumor family? Int J Surg Pathol. 2008;16:16–20.PubMedCrossRef Righi A, Dimosthenous K, Rosai J. PEComa: another member of the MiT tumor family? Int J Surg Pathol. 2008;16:16–20.PubMedCrossRef
21.
Zurück zum Zitat Torii I, Kondo N, Takuwa T, et al. Perivascular epithelioid cell tumor of the rib. Virchows Arch. 2008;452:697–702.PubMedCrossRef Torii I, Kondo N, Takuwa T, et al. Perivascular epithelioid cell tumor of the rib. Virchows Arch. 2008;452:697–702.PubMedCrossRef
22.
23.
Zurück zum Zitat Rakheja R, Abikhzer G, Alabed YZ, Nahal A, Lisbona R. The appearance of osseous PEComa on F-18 FDG PET/CT. Clin Nucl Med. 2012;37:190–2.PubMedCrossRef Rakheja R, Abikhzer G, Alabed YZ, Nahal A, Lisbona R. The appearance of osseous PEComa on F-18 FDG PET/CT. Clin Nucl Med. 2012;37:190–2.PubMedCrossRef
24.
Zurück zum Zitat Marrari A, Hornick JL, Butrynski JE, et al. Malignant perivascular epithelioid cell tumors (PEComas): a retrospective analysis of 24 patients to define clinical history and response to therapy. Paper presented at: 2011 Combined Meeting of the Connective Tissue Oncology Society and the Musculoskeletal Tumor Society; 2011 Oct. 26-29; Chicago, Illinois. Marrari A, Hornick JL, Butrynski JE, et al. Malignant perivascular epithelioid cell tumors (PEComas): a retrospective analysis of 24 patients to define clinical history and response to therapy. Paper presented at: 2011 Combined Meeting of the Connective Tissue Oncology Society and the Musculoskeletal Tumor Society; 2011 Oct. 26-29; Chicago, Illinois.
Metadaten
Titel
Primary perivascular epithelioid cell neoplasm (PEComa) of bone: Report of two cases and review of the literature
verfasst von
Nicholas M. Desy
Mitchell Bernstein
Ayoub Nahal
Mohamed Aziz
Samuel Kenan
Robert E. Turcotte
Leonard B. Kahn
Publikationsdatum
01.11.2012
Verlag
Springer-Verlag
Erschienen in
Skeletal Radiology / Ausgabe 11/2012
Print ISSN: 0364-2348
Elektronische ISSN: 1432-2161
DOI
https://doi.org/10.1007/s00256-012-1479-1

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