{"paper_id":"907ebad0-b7f8-4902-96b2-f7bd3b527f56","body_text":"INTRODUCTION\nSince Sampson first described the development of ovarian\ncarcinoma in endometriosis in 1925, numerous case re-\nports have documented the development of malignancies\nfrom endometriosis (1-3). Most of the malignant neoplasms\nare adenocarcinomas of endometrioid and clear cell types, and\nextrauterine endometrial stromal sarcoma is an extremely rare\ntumor (1-7). Recently, Yantiss et al. (7) reported only one\nendometrial stromal sarcoma among seventeen malignant\nneoplasms arising in gastrointestinal endometriosis. We found\nonly six cases of endometrial stromal sarcoma of the intesti-\nnal tract arising in endometriosis in English literatures (1,\n6-8). A case of endometrial stromal sarcoma of the sigmoid\ncolon arising in endometriosis is described with a review of\nliteratures. \nCASE REPORT\nThe patient was 48-yr-old female who complained of dif-\nficult defecation and tenesmus. She had a history of subtotal\nhysterectomy for myoma uteri three years before, followed\nby total hysterectomy and salpingo-oophorectomy due to\nendometriosis of the left ovary the following year. On endo-\nscopic examination, a polypoid lesion was found at the recto-\nsigmoid area. Under the diagnosis of gastrointestinal stro-\nmal tumor (GIST), segmental resection of the sigmoid colon\nwith regional lymph node dissection was performed. On\noperation, tumor invasions to the urinary bladder and ureter\nwere noted. Multiple nodules on the mesentery and lymph\nnodes enlargement were also found. Grossly, resected sigmoid\ncolon had multinodular masses of 1-3 cm in size, involving\nfrom the mucosa to serosa (Fig. 1). Histologically, the lesion\nwas characterized by round or tongue-like multinodular growth\nof closely packed plump spindle cells (Fig. 2) with prominent\nspiral arterioles (Fig. 3). The cellular atypism was minimal,\nand mitoses were rare. Rod- or star-shaped hyaline plaques\nwere found. At the periphery and surface of the lesions, nor-\nmal-appearing proliferative phase endometrial glands and\nstroma, corresponding to benign endometriosis, were present\n(Fig. 4).There were no periglandular stromal condensations\nthat is charateristic of m llerian adenosarcoma. On immuno-\nhistochemical stain, the tumor cells were positive for vimentin,\nbut negative for smooth muscle actin, S-100 protein, CD34,\nor c-kit (CD117). Most of the cells were also strongly positive\nfor progesterone receptor, and weakly positive for estrogen\nreceptor. Ki-67 labelling was low. Mesenteric nodules rep-\nresented metastatic stromal sarcoma. The patient was com-\nplicated by postoperative disseminated intravascular coagulation\n(DIC), which was successfully managed and recovered. The\npatient was alive four months after the resection with no\nevidence of recurrence.\nHyun-Yee Cho*, Min-Kyung Kim, \nSeong-Jin Cho, Jung-Won Bae\n�\n, \nInsun Kim \nDepartment of Pathology, Gachon Medical School*,\nInchon; Department of Surgery\n�\n, Department of\nPathology, Korea University Medical College,\nSeoul, KoreaAddress for correspondence\nInsun Kim, M.D.\nDepartment of Pathology, Korea University Medical\nCollege, 126-1, 5-ga, Anam-dong, Sungbuk-gu,\nSeoul 136-705, Korea\nTel : +82.2-920-6373, Fax : +82.2-923-1340\nE-mail : iskim@korea.ac.kr\n412\nJ Korean Med Sci 2002; 17: 412-4\nISSN 1011-8934\nCopyright � The Korean Academy\nof Medical Sciences\nEndometrial Stromal Sarcoma of the Sigmoid Colon Arising in\nEndometriosis \n: A Case Report with a Review of Literatures\nMost of malignant tumors arising in ovarian and extraovarian endometriosis are\ncarcinomas. Mixed mullerian tumor and endometrial stromal sarcoma arising in\nintestinal endometriosis are rarely described, but its clinicopathologic features have\nnot been well characterized. Here we report a case of endometrial stromal sar-\ncoma of the sigmoid colon arising in endometriosis with a review of six addition-\nal cases of endometrial stromal sarcoma arising in intestinal endometriosis found\nin English literatures. The patients ranged in age from 36 to 64 yr. Presenting\nsymptoms were pain, bloody diarrhea, and tenesmus. Some patients had a pre-\nvious history of endometriosis. Most of the tumors arose in the rectosigmoid\ncolon. The histologic features were the same as their uterine counterpart. No death\nof disease had been reported. This rare tumor should not be confused with gas-\ntrointestinal stromal tumor clinically and histologically. \nKey Words : Endometriosis; Sarcomas, Endometrial Stromal; Intestines\nReceived : 11 December 2000\nAccepted : 1 June 2001\n..\n\nDISCUSSION\nIn 1925, Sampson defined an entity as originating from\nendometriosis when it satisfied three criteria: clear examples\nof endometriosis in close proximity to the tumor, no other\nprimary site of malignancy, and histological appearance com-\npatible with an origin from endometriosis (1). Many cases of\nmalignant neoplasms arising in ovarian and extraovarian\nendometriosis have been described thereafter (1-3). The fre-\nquency of malignant transformation of endometriosis is\nunknown, but it is estimated that up to 1% of women with\nendometriosis will develop endometriosis-associated neoplasm\n(2). Nearly 75% of the reported tumors arose in the ovary,\nbut 21.3% appear in extragonadal sites (2, 4). The majority\nEndometrial Stromal Sarcoma of the Colon 413\nFig. 1. Grossly, the resected sigmoid colon had multinodular\nmasses involving the wall from the mucosa to the serosa.\nFig. 2. The tumor is characterized by tongue-like multinodular\nproliferation of closely packed spindle cells (H&E, ×12.5).\nFig. 3. The tumor was composed of short fascicles or sheets of\nmonotonous plump spindle cells with abundant arterioles. A\nperivascular whorl arrangement is prominent (H&E,×200).\nFig. 4. At the periphery and surface of the tumor, glands and\nstroma, corresponding to benign endometriosis, are present.\nThere are no periglandular stromal condensation that is charac-\nteristic of mullerian adenosarcoma (H&E,×40).\nKUH 00-6934\n..\n\nof malignant tumors arising from endometriosis were ade-\nnocarcinomas, particularly endometrioid and clear cell car-\ncinomas, but stromal sarcoma and mixed m llerian tumors\ncan be developed (1-9). The same kinds of neoplasms have been\nreported in endometriosis of the intestinal tract (1, 6-9). \nRecently, Yantiss et al. (7) described a large series of neoplas-\ntic and pre-neoplastic lesions of gastrointestinal endometriosis:\namong the seventeen cases, endometrial stromal sarcoma was\nonly one and endometrioid adenocarcinomas were eight with\nfour m llerian adenosarcomas, one endometrioid adenofi-\nbroma of borderline malignancy, one endometrial atypical\nhyperplasia, and one adenocarcinoma in situ. Using Sampson's\ncriteria, we found only five additional cases of endometrial\nstromal sarcoma arising from intestinal endometriosis in\nEnglish literatures (1, 6-9). \nSeven cases including our case were analyzed to define the\nclinical and pathologic characteristics of this tumor. The seven\npatients ranged in age from 36 to 64 yr (average 51 yr). The\ntwo younger patients were nulliparous, and as for the other\nthree, parity was not stated. Presenting symptoms werevari-\nable; pain in two, bloody diarrhea in one and tenesmus in\none. Two patients had undergone previous surgical proce-\ndures aimed at treating the endometriosis (8). Among seven\ncases, five tumors were located in the rectosigmoid area. The\nrectosigmoid is an area of bowel having the highest incidence\nof endometriosis (4). Like their endometriotic precursors, the\ntumors occurred at various locations in the bowel wall. The\npathologic features of the tumors were very similar and were\nvirtually the same as their uterine counterpart. Although data\nconcerning the survival were difficult to assess because the\nfollow-up had generally been limited with only four cases with\na documentation of follow-up for five or more years, no death\nof disease has been reported.\nThe endometrial stromal sarcoma of the intestinal tract\narising in endometriosis should always be included in the\ndifferential diagnosis of small round cell or spindle cell tumors\nof the intestinal tract of female patients. The distinction from\nGIST should be considered, because the GIST is the most\ncommon mesenchymal tumor occurring in the intestinal tract\nand is managed differently from endometrial stromal sarco-\nma. Especially, if the underlying benign endometriosis is\nobscured, endometrial stromal sarcoma may be confused with\nGIST. In contrast to endometrial stromal sarcoma, most GISTs\ntend to be well-circumscribed with broad and pushing bor-\nders. The presence of characteristic morphologic features such\nas invasive “tongues” of tumor at the periphery of the neoplasm,\nshort fascicles or sheets of monotonous plump spindle cells,\nand prominent arterioles should raise the suspicion of an\nendometrial stromal sarcoma. Immunopositivity for c-kit in\nGIST may be useful to distinguish these two entities (10).\nM llerian adenosarcoma with sarcomatous overgrowth should\nalso be included in differential diagnosis. Periglandular cel-\nlular stromal condensation, polypoid extensions of cellular\nstroma into the glandular lumens, and various m llerian types\nof glandular epithelium favor the diagnosis of m llerian\nadenosarcoma with sarcomatous overgrowth. In our case, the\npresence of endometrial glands without periglandular stromal\ncondensation and the immunohistochemical characteristics\nsuch as negative stainings for smooth muscle actin, S-100\nprotein, CD34, and c-kit were not consistent with GIST or\nm llerian adenosarcoma with sarcomatous overgrowth. \nThe present case was postoperatively complicated by DIC.\nA case of metastatic endometrial stromal sarcoma to the right\nventricle associated with DIC has been described by Matsumoto\net al. (11). Although the mechanism of DIC is not clear and\nits occurrence is rare, the possibility of DIC should be con-\nsidered in the management of the patient with endometrial\nstromal sarcoma.   \nREFERENCES\n1. Scully RE, Richardson GS, Barlow JF. The development of malig-\nnancy in endometriosis. Clin Obstet Gynecol 1966; 9: 384-411.\n2. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasm arising in\nendometriosis. Obstet Gynecol 1990; 75: 1023-8.\n3. Hyman MP. Extraovarian endometrioid carcinoma: review of the\nliterature and report of two cases with unusual features. Am J Clin\nPathol 1977; 68: 522-7.\n4. Mostoufizadeh M, Scully RE. Malignant tumors arising in endometrio-\nsis. Clin Obstet Gynecol 1980; 23: 951-63.\n5. Palladino VS, Trousdell M. Extra-uterine mullerian tumors : a review\nof the literature and report of a case. Cancer 1969; 23: 1413-22.\n6. Chang KL, Crabtree GS, Lim-Tan SK, Kepson RL, Hendrickson\nMR. Primary extrauterine endometrial stromal neoplasms: a clini-\ncopathologic study of 20 cases and a review of the literature. Int J\nGynecol Pathol 1993; 12: 282-96.\n7. Yantiss RK, Clement PB, Young RH. Neoplastic and pre-neoplastic\nchanges in gastrointestinal endometriosis. A study of 17 cases. Am J\nSurg Pathol 2000; 24: 513-24.\n8. Biocchi G, Kavanagh JJ, Wharton JT. Endometrioid stromal sarco-\nmas arising from ovarian and extraovarian endometriosis: Report\nof two cases and review of the literature. Gynecol Oncol 1990; 36:\n147-51.\n9. Ferraro LR, Hetz H, Carter H. Malignant endometriosis-Pelvic endo-\nmetriosis complicated by polypoid endometrioma of the colon and\nendometriotic sarcoma; report of a case and review of the literature.\nObstet Gynecol 1956; 7: 32-9.\n10. Sarlomo-Rikara M, Kovatich AJ, Barsevicius A, Miettinen M.\nCD117: A sensitive marker for gastrointestinal stromal tumors that\nis more specific than CD34. Mod Pathol 1998; 11: 728-34.\n11. Matsumoto N, Ohteki H, Doi K, Sakai M, Furugawa K. Metastatic\nendometrial sarcoma of the right ventricular outflow tract associated\nwith disseminated intravascular coagulopathy. Kyobu Geka 1999;\n52: 401-4.\n414 H.-Y. Cho, M.-K. Kim, S.-J. Cho, et al.","source_license":"CC0","license_restricted":false}