PEComa of the Upper Extremity: A Unique Case and Description of an Initial Response to Neoadjuvant Chemotherapy

Osei, D. A.; Alvandi, F.; Brooks, J. S.; Ogilvie, C. M.

doi:https://doi.org/10.1155/2007/53056

Sarcoma

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Case Report | Open Access

Volume 2007 | Article ID 053056 | https://doi.org/10.1155/2007/53056

PEComa of the Upper Extremity: A Unique Case and Description of an Initial Response to Neoadjuvant Chemotherapy

D. A. Osei,¹F. Alvandi,²J. S. Brooks,²and C. M. Ogilvie³

Academic Editor: Raphael E. Pollock

Received02 Sept 2005

Revised01 Jun 2007

Accepted15 Aug 2007

Published16 Dec 2007

Abstract

Purpose: Tumors of the perivascular epithelial cell tumor (PEComa), first described in 1992, represent a rare soft tissue neoplasm of varying malignant potential. Cases of PEComa have been previously described in a few somatic and visceral sites, most notably in the gastrointestinal tract, genitourinary tract, and one extremity case in the thigh. To date, most malignant cases of PEComa have been resistant to chemotherapy, and as such, an appropriate therapy is not known. Case report: Here we describe the first case of PEComa of the upper extremity. Open biopsy revealed a high-grade malignant lesion, and the patient subsequently underwent both neoadjuvant therapy with doxorubicin, ifosfamide and mensa, and radiation therapy prior to wide surgical resection. After six cycles of chemotherapy, the tumor underwent an 80% reduction in size. Subsequent neoadjuvant radiation therapy of 5000 cGy did not further reduce the size of the tumor. Following limb sparing radical resection, pathology showed 20% necrosis within a high-grade malignant lesion. Twenty one months after beginning treatment, the patient shows no sign of local recurrence, but metastatic disease was confirmed after resection of a lung nodule. Conclusion: Given the favorable albeit partial response seen in this patient, the course of therapy outlined here may represent a good starting point for neoadjuvant treatment in a tumor with a historically bleak prognosis. In addition, the diagnosis of PEComa must now be entertained in the differential diagnosis of upper extremity soft tissue sarcoma.

References

F. Bonetti, M. Pea, and G. Martignoni et al., “Clear cell (sugar) tumor of the lung is a lesion strictly related to angiomyolipoma: the concept of a family of lesions characterized be the presence of the perivascular epitheliod cells (PEC),” Pathology, vol. 26, no. 3, pp. 230–236, 1992.
View at: Publisher Site | Google Scholar
F. Bonetti, G. Martignoni, and C. Colato et al., “Abdominopelvic sarcoma of perivascular epitheliod cells. Report of four cases in young women, one with tuberous sclerosis,” Modern Pathology, vol. 14, no. 6, pp. 563–568, 2001.
View at: Publisher Site | Google Scholar
F. Birkhaeuser, C. Ackerman, and T. Flueckiger et al., “First description of a PEComa (Perivascular Epitheliod Cell tumor) of the colon: report of a case and review of the literature,” Diseases of the Colon and Rectum, vol. 47, no. 10, pp. 1734–1737, 2004.
View at: Publisher Site | Google Scholar
A. L. Folpe, Z. D. Goodman, and K. G. Ishak et al., “Clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres,” The American Journal of Surgical Pathology, vol. 24, no. 9, pp. 1239–1246, 2000.
View at: Publisher Site | Google Scholar
L. A. Greene, S. L. Mount, and A. R. Schned et al., “Recurrent perivascular epitheliod cell tumor of the uterus (PEComa): an immunohistochemical study and review of literature,” Gynecologic Oncology, vol. 90, no. 3, pp. 677–681, 2003.
View at: Publisher Site | Google Scholar
H. Rigby, Y. Weiming, and M. H. Schmidt et al., “Lack of a response of a metastatic renal perivascular epitheliod cell tumor (PEComa) to successive courses of DTIC based-therapy and Imatinib Mesylate,” Pediatric Blood & Cancer, vol. 44, pp. 1–5, 2005.
View at: Google Scholar
N. L. Lehman et al., “Malignant PEComa of the Skull Base,” The American Journal of Surgical Pathology, vol. 28, pp. 1230–1232, 2004.
View at: Google Scholar
A. L. Folpe, J. K. McKenney, and L. Zhiming et al., “Clear cell myomelanocytic tumor of the thigh: report of a unique case,” The American journal of surgical pathology, vol. 26, no. 6, pp. 809–812, 2002.
View at: Publisher Site | Google Scholar
C. D.-M. Fletcher, K. K. Unni, and F. Mertens, Eds., World Health Organization Classification of Tumours: Pathology and Genetics Tumours of Soft Tissue and Bone, IARC, Lyon, France, 2002.
View at: Google Scholar
E. S. Cibas, G. A. Goss, M. H. Kulke, C. D.-M. Fletcher, and F. R.-C. Path, “Malignant epithelioid angiomyolipoma (sarcoma ex angiomyolipoma) of the kidney: a case report and review of the literature,” The American Journal of Surgical Pathology, vol. 25, no. 1, pp. 121–126, 2001.
View at: Publisher Site | Google Scholar
N. Takahashi, R. Kitahara, Y. Hishimoto, A. Ohguro, Y. Hashimoto, and T. Suzuki, “Malignant transformation of renal angiomyolipoma,” International Journal of Urology, vol. 10, no. 5, pp. 271–273, 2003.
View at: Publisher Site | Google Scholar
Sarcoma Meta-analysis Collaboration, “Adjuvant chemotherapy for localized respectable soft-tissue sarcoma of adults: meta-analysis of individual data,” Lancet, vol. 350, no. 9092, pp. 1647–1654, 1997.
View at: Publisher Site | Google Scholar
S. Frustaci, F. Gherlinzoni, and A. De Paoli et al., “Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: Results of the italian randomized cooperative trial,” Journal of Clinical Oncology, vol. 19, no. 5, pp. 1238–1247, 2001.
View at: Google Scholar
S. R. Grobmyer, R. G. Maki, and G. D. Demetri et al., “Neoadjuvant chemotherapy for primary high grade extremity soft tissue sarcoma,” Annals of Oncology, vol. 15, no. 11, pp. 1667–1672, 2004.
View at: Publisher Site | Google Scholar
F. C. Eilber, G. Rosen, and J. Eckardt et al., “Treatment induced pathologic necrosis: a predictor of local recurrence and survival in patients receiving neoadjuvant therapy for high grade extremity soft tissue sarcomas,” Journal of clinical oncology, vol. 19, pp. 3203–3209, 2001.
View at: Google Scholar
T. F. DeLaney, I. J. Spiro, and H. D. Suit et al., “Neoadjuvant chemotherapy and radiotherapy for large extremity soft tissue sarcomas,” International Journal of Radiation Oncology, Biology, Physics, vol. 56, no. 4, pp. 1117–1127, 2003.
View at: Publisher Site | Google Scholar
M. Scurr and I. Judson et al., “ Neoadjuvant and adjuvant therapy for extremity soft tissue sarcomas,” Hematology/Oncology Clinics of North America, vol. 19, no. 3, pp. 489–500, 2005.
View at: Publisher Site | Google Scholar
K. Antman, “New biology and therapies in soft tissue sarcomas,” Biomedicine and Pharmacotherapy, vol. 55, no. 9-10, pp. 553–557, 2001.
View at: Publisher Site | Google Scholar
L. Y. Dirix and A. T. Van Oosterom, “Soft tissue sarcoma in adults,” Current Opinion in Oncology, vol. 11, no. 4, pp. 285–295, 1999.
View at: Publisher Site | Google Scholar

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Copyright © 2007 D. A. Osei et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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