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Journal of Clinical Oncology, Vol 23, No 4 (February 1), 2005: pp. 910-912 © 2005 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.03.096
Small Blue Cell Tumors of the RectumCASE 1. Ewing’s Sarcoma of the RectumDepartment of Medical Oncology, Sydney Cancer Centre, Royal Prince Alfred Hospital, Sydney, Australia
Department of Anatomical Pathology, Concord Repatriation General Hospital, Sydney, Australia A previously healthy 53-year-old male house painter of Greek origin presented to his local doctor after a single episode of bright rectal bleeding. Additional questioning determined that he had experienced a sensation of heaviness in the rectum for several months. Sigmoidoscopy revealed a fungating, ulcerated posterior wall rectal mass. Treatment necessitated a low anterior resection with formation of a temporary ileostomy. The tumor extended through the full thickness of the rectal wall and to within 7 mm of the resection margin. Histopathology showed an undifferentiated small round blue cell tumor (Fig 1, arrows) with high mitotic rate and extensive areas of necrosis (Fig 2A). Immunoperoxidase stains were positive for MIC-2 (CD99; Fig 2B) and vimentin. There was focal keratin positivity with CAM 5.2. Stains for keratin AE1/AE3, S-100, chromogranin, desmin, and smooth muscle actin were negative. Neuron-specific enolase staining was nonspecific. The tumor cells contained abundant glycogen demonstrated by the periodic acid Schiff stain before and after diastase treatment (Figs. 3A and 3B). No rosette formation was seen. The histologic appearance and immunoperoxidase profile supported a diagnosis of extraosseous Ewing’s sarcoma (EOE). Additional staging using blood results; computed tomography of chest, abdomen, and pelvis; and a whole-body bone scan showed no evidence of disseminated malignancy.
Because of the full thickness rectal wall involvement, the patient was deemed to be high risk for recurrence and was treated with 25 fractions of radiotherapy to the pelvis and four cycles of adjuvant chemotherapy with cyclophosphamide, vincristine, and doxorubicin. On completion of the adjuvant treatment, the stoma was reversed without complication. Twelve months after initial diagnosis, the patient was asymptomatic; however, routine restaging with computed tomography scans revealed multiple widespread pulmonary metastases. He was treated with additional chemotherapy but, unfortunately, subsequent metastases occurred, including brain involvement. The patient was eventually given supportive care and died, almost 2 years after his initial diagnosis. A MEDLINE and CANCERLIT search reveals no reported cases of Ewing’s sarcoma of the rectum. EOE is a rare malignant soft tissue tumor, seen more commonly in children and young adults.1,2 It was initially reported in 1969, with description of a paravertebral soft tissue mass having histologic appearance of Ewing’s sarcoma.3 Since then there have been fewer than 300 reported cases of EOE.4 EOEs are generally considered to be part of the spectrum of the Ewing’s family of tumors, which includes osseous Ewing’s sarcoma (OES), EOE, and primitive neuroectodermal tumors (PNET). These tumors all show similar histologic features of densely packed, small, round blue cells without distinct cell borders.5 The majority have a reciprocal chromosomal translocation t11:22 (q24:q12).1,2,6–8 MIC-2 antibody (CD99) is expressed by both OES and PNET. PNETs typically show evidence of neuroepithelial differentiation with Homer Wright rosettes and NSE positivity, whereas OES lacks rosettes and contains cytoplasmic glycogen.5,9 The most common primary sites of EOE are the extremities, paravertebral region, retroperitoneum, and chest wall.2,4,6,10–12 Metastases most commonly occur in the lungs and bone1,4,10–12 and tend to occur early.4 The Intergroup Rhabdomyosarcoma Study reported that only 130 (5%) of 2,792 patients enrolled onto trials undertaken from 1972 to 1991 had EOE.12 All were younger than 21 years old and 54% were male. Primary tumor sites included trunk, 31%; extremity, 26%; head and neck, 18%; and retroperitoneum/pelvis, 16%. Twelve percent had metastases at diagnosis. All patients received combination chemotherapy and the majority also received surgery and radiotherapy. A complete response was achieved in 82%, with 10-year survival 62% to 77%. Best prognosis occurred with head and neck primaries. In another report, patients with localized EOE had a 5-year actuarial disease-specific survival of 46%, with a median survival of 8 months for metastatic disease.13 It was suggested that the clinicopathological features of EOE were different than that of OES, with a higher risk of relapse and a poorer response to chemotherapy. To our knowledge, there is no other reported case of EOE of the rectum. For most EOE, treatment consists of surgery, followed by combination chemotherapy and often radiotherapy. Authors’ Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Kaspers GJ, Kamphorst W, van de Graaff M, et al: Primary spinal epidural extraosseous Ewing’s sarcoma. Cancer 68:648-654, 1991[CrossRef][Medline] 2. Charny CK, Glick RD, Genega EM, et al: Ewing’s sarcoma/primitive neuroectodermal tumor of the ureter: A case report and review of the literature. J Pediatr Surg 35:1356-1358, 2000[CrossRef][Medline] 3. Tefft M, Vawter GF, Mitus A: Paravertebral "round cell" tumors in children. Radiology 92:1501-1509, 1969[Medline] 4. Angervall L, Enzinger FM: Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer 36:240-251, 1975[CrossRef][Medline] 5. Schmidt D, Herrmann C, Jurgens H, et al: Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing’s sarcoma: A report from the Kiel Pediatric Tumor Registry. Cancer 68:2251-2259, 1991[CrossRef][Medline] 6. Newton WA Jr, Soule EH, Hamoudi AB, et al: Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: Clinicopathologic correlation. J Clin Oncol 6:67-75, 1988[Abstract] 7. Toh KL, Tan PH, Cheng WS: Primary extraskeletal Ewing’s sarcoma of the external genitalia. J Urol 162:159-160, 1999[CrossRef][Medline] 8. Sheaff M, McManus A, Scheimberg I, et al: Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. Am J Surg Pathol 21:461-468, 1997[CrossRef][Medline] 9. Ambros IM, Ambros PF, Strehl S, et al: MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors: Evidence for a common histogenesis of Ewing’s sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer 67:1886-1893, 1991[CrossRef][Medline] 10. Soule EH, Newton W Jr, Moon TE, et al: Extraskeletal Ewing’s sarcoma: A preliminary review of 26 cases encountered in the Intergroup Rhabdomyosarcoma Study. Cancer 42:259-264, 1978[CrossRef][Medline] 11. Rud NP, Reiman HM, Pritchard DJ, et al: Extraosseous Ewing’s sarcoma: A study of 42 cases. Cancer 64:1548-1553, 1989[CrossRef][Medline]
12. Raney RB, Asmar L, Newton WA Jr, et al: Ewing’s sarcoma of soft tissues in childhood: A report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 15:574-582, 1997
13. Ahmad SA, Patel SR, Ballo MT, et al: Extraosseous osteosarcoma: Response to treatment and long-term outcome. J Clin Oncol 20:521-527, 2002
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Copyright © 2005 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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