Endometrial Stromal Sarcoma of the Sigmoid Colon Arising in Endometriosis :A Case Report with a Review of Literatures

Since Sampson first described the development of ovarian carcinoma in endometriosis in 1925, numerous case re- ports have documented the development of malignancies from endometriosis (1-3). Most of the malignant neoplasms are adenocarcinomas of endometrioid and clear cell types, and extrauterine endometrial stromal sarcoma is an extremely rare tumor (1-7). Recently, Yantiss et al. (7) reported only one endometrial stromal sarcoma among seventeen malignant neoplasms arising in gastrointestinal endometriosis. We found only six cases of endometrial stromal sarcoma of the intesti- nal tract arising in endometriosis in English literatures (1, 6-8). A case of endometrial stromal sarcoma of the sigmoid colon arising in endometriosis is described with a review of literatures. CASE REPORT The patient was 48-yr-old female who complained of dif- ficult defecation and tenesmus. She had a history of subtotal hysterectomy for myoma uteri three years before, followed by total hysterectomy and salpingo-oophorectomy due to endometriosis of the left ovary the following year. On endo- scopic examination, a polypoid lesion was found at the recto- sigmoid area. Under the diagnosis of gastrointestinal stro- mal tumor (GIST), segmental resection of the sigmoid colon with regional lymph node dissection was performed. On operation, tumor invasions to the urinary bladder and ureter were noted. Multiple nodules on the mesentery and lymph nodes enlargement were also found. Grossly, resected sigmoid colon had multinodular masses of 1-3 cm in size, involving from the mucosa to serosa (Fig. 1). Histologically, the lesion was characterized by round or tongue-like multinodular growth of closely packed plump spindle cells (Fig. 2) with prominent spiral arterioles (Fig. 3). The cellular atypism was minimal, and mitoses were rare. Rod- or star-shaped hyaline plaques were found. At the periphery and surface of the lesions, nor- mal-appearing proliferative phase endometrial glands and stroma, corresponding to benign endometriosis, were present (Fig. 4).There were no periglandular stromal condensations that is charateristic of m llerian adenosarcoma. On immuno- histochemical stain, the tumor cells were positive for vimentin, but negative for smooth muscle actin, S-100 protein, CD34, or c-kit (CD117). Most of the cells were also strongly positive for progesterone receptor, and weakly positive for estrogen receptor. Ki-67 labelling was low. Mesenteric nodules rep- resented metastatic stromal sarcoma. The patient was com- plicated by postoperative disseminated intravascular coagulation (DIC), which was successfully managed and recovered. The patient was alive four months after the resection with no evidence of recurrence. Hyun-Yee Cho*, Min-Kyung Kim, Seong-Jin Cho, Jung-Won Bae � , Insun Kim Department of Pathology, Gachon Medical School*, Inchon; Department of Surgery � , Department of Pathology, Korea University Medical College, Seoul, KoreaAddress for correspondence Insun Kim, M.D. Department of Pathology, Korea University Medical College, 126-1, 5-ga, Anam-dong, Sungbuk-gu, Seoul 136-705, Korea Tel : +82.2-920-6373, Fax : +82.2-923-1340 E-mail : [email protected] 412 J Korean Med Sci 2002; 17: 412-4 ISSN 1011-8934 Copyright � The Korean Academy of Medical Sciences Endometrial Stromal Sarcoma of the Sigmoid Colon Arising in Endometriosis : A Case Report with a Review of Literatures Most of malignant tumors arising in ovarian and extraovarian endometriosis are carcinomas. Mixed mullerian tumor and endometrial stromal sarcoma arising in intestinal endometriosis are rarely described, but its clinicopathologic features have not been well characterized. Here we report a case of endometrial stromal sar- coma of the sigmoid colon arising in endometriosis with a review of six addition- al cases of endometrial stromal sarcoma arising in intestinal endometriosis found in English literatures. The patients ranged in age from 36 to 64 yr. Presenting symptoms were pain, bloody diarrhea, and tenesmus. Some patients had a pre- vious history of endometriosis. Most of the tumors arose in the rectosigmoid colon. The histologic features were the same as their uterine counterpart. No death of disease had been reported. This rare tumor should not be confused with gas- trointestinal stromal tumor clinically and histologically. Key Words : Endometriosis; Sarcomas, Endometrial Stromal; Intestines Received : 11 December 2000 Accepted : 1 June 2001 ..

In 1925, Sampson defined an entity as originating from endometriosis when it satisfied three criteria: clear examples of endometriosis in close proximity to the tumor, no other primary site of malignancy, and histological appearance com- patible with an origin from endometriosis (1). Many cases of malignant neoplasms arising in ovarian and extraovarian endometriosis have been described thereafter (1-3). The fre- quency of malignant transformation of endometriosis is unknown, but it is estimated that up to 1% of women with endometriosis will develop endometriosis-associated neoplasm (2). Nearly 75% of the reported tumors arose in the ovary, but 21.3% appear in extragonadal sites (2, 4). The majority Endometrial Stromal Sarcoma of the Colon 413 Fig. 1. Grossly, the resected sigmoid colon had multinodular masses involving the wall from the mucosa to the serosa. Fig. 2. The tumor is characterized by tongue-like multinodular proliferation of closely packed spindle cells (H&E, ×12.5). Fig. 3. The tumor was composed of short fascicles or sheets of monotonous plump spindle cells with abundant arterioles. A perivascular whorl arrangement is prominent (H&E,×200). Fig. 4. At the periphery and surface of the tumor, glands and stroma, corresponding to benign endometriosis, are present. There are no periglandular stromal condensation that is charac- teristic of mullerian adenosarcoma (H&E,×40). KUH 00-6934 .. of malignant tumors arising from endometriosis were ade- nocarcinomas, particularly endometrioid and clear cell car- cinomas, but stromal sarcoma and mixed m llerian tumors can be developed (1-9). The same kinds of neoplasms have been reported in endometriosis of the intestinal tract (1, 6-9). Recently, Yantiss et al. (7) described a large series of neoplas- tic and pre-neoplastic lesions of gastrointestinal endometriosis: among the seventeen cases, endometrial stromal sarcoma was only one and endometrioid adenocarcinomas were eight with four m llerian adenosarcomas, one endometrioid adenofi- broma of borderline malignancy, one endometrial atypical hyperplasia, and one adenocarcinoma in situ. Using Sampson's criteria, we found only five additional cases of endometrial stromal sarcoma arising from intestinal endometriosis in English literatures (1, 6-9). Seven cases including our case were analyzed to define the clinical and pathologic characteristics of this tumor. The seven patients ranged in age from 36 to 64 yr (average 51 yr). The two younger patients were nulliparous, and as for the other three, parity was not stated. Presenting symptoms werevari- able; pain in two, bloody diarrhea in one and tenesmus in one. Two patients had undergone previous surgical proce- dures aimed at treating the endometriosis (8). Among seven cases, five tumors were located in the rectosigmoid area. The rectosigmoid is an area of bowel having the highest incidence of endometriosis (4). Like their endometriotic precursors, the tumors occurred at various locations in the bowel wall. The pathologic features of the tumors were very similar and were virtually the same as their uterine counterpart. Although data concerning the survival were difficult to assess because the follow-up had generally been limited with only four cases with a documentation of follow-up for five or more years, no death of disease has been reported. The endometrial stromal sarcoma of the intestinal tract arising in endometriosis should always be included in the differential diagnosis of small round cell or spindle cell tumors of the intestinal tract of female patients. The distinction from GIST should be considered, because the GIST is the most common mesenchymal tumor occurring in the intestinal tract and is managed differently from endometrial stromal sarco- ma. Especially, if the underlying benign endometriosis is obscured, endometrial stromal sarcoma may be confused with GIST. In contrast to endometrial stromal sarcoma, most GISTs tend to be well-circumscribed with broad and pushing bor- ders. The presence of characteristic morphologic features such as invasive “tongues” of tumor at the periphery of the neoplasm, short fascicles or sheets of monotonous plump spindle cells, and prominent arterioles should raise the suspicion of an endometrial stromal sarcoma. Immunopositivity for c-kit in GIST may be useful to distinguish these two entities (10). M llerian adenosarcoma with sarcomatous overgrowth should also be included in differential diagnosis. Periglandular cel- lular stromal condensation, polypoid extensions of cellular stroma into the glandular lumens, and various m llerian types of glandular epithelium favor the diagnosis of m llerian adenosarcoma with sarcomatous overgrowth. In our case, the presence of endometrial glands without periglandular stromal condensation and the immunohistochemical characteristics such as negative stainings for smooth muscle actin, S-100 protein, CD34, and c-kit were not consistent with GIST or m llerian adenosarcoma with sarcomatous overgrowth. The present case was postoperatively complicated by DIC. A case of metastatic endometrial stromal sarcoma to the right ventricle associated with DIC has been described by Matsumoto et al. (11). Although the mechanism of DIC is not clear and its occurrence is rare, the possibility of DIC should be con- sidered in the management of the patient with endometrial stromal sarcoma.

1. Scully RE, Richardson GS, Barlow JF. The development of malig- nancy in endometriosis. Clin Obstet Gynecol 1966; 9: 384-411. 2. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasm arising in endometriosis. Obstet Gynecol 1990; 75: 1023-8. 3. Hyman MP. Extraovarian endometrioid carcinoma: review of the literature and report of two cases with unusual features. Am J Clin Pathol 1977; 68: 522-7. 4. Mostoufizadeh M, Scully RE. Malignant tumors arising in endometrio- sis. Clin Obstet Gynecol 1980; 23: 951-63. 5. Palladino VS, Trousdell M. Extra-uterine mullerian tumors : a review of the literature and report of a case. Cancer 1969; 23: 1413-22. 6. Chang KL, Crabtree GS, Lim-Tan SK, Kepson RL, Hendrickson MR. Primary extrauterine endometrial stromal neoplasms: a clini- copathologic study of 20 cases and a review of the literature. Int J Gynecol Pathol 1993; 12: 282-96. 7. Yantiss RK, Clement PB, Young RH. Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis. A study of 17 cases. Am J Surg Pathol 2000; 24: 513-24. 8. Biocchi G, Kavanagh JJ, Wharton JT. Endometrioid stromal sarco- mas arising from ovarian and extraovarian endometriosis: Report of two cases and review of the literature. Gynecol Oncol 1990; 36: 147-51. 9. Ferraro LR, Hetz H, Carter H. Malignant endometriosis-Pelvic endo- metriosis complicated by polypoid endometrioma of the colon and endometriotic sarcoma; report of a case and review of the literature. Obstet Gynecol 1956; 7: 32-9. 10. Sarlomo-Rikara M, Kovatich AJ, Barsevicius A, Miettinen M. CD117: A sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol 1998; 11: 728-34. 11. Matsumoto N, Ohteki H, Doi K, Sakai M, Furugawa K. Metastatic endometrial sarcoma of the right ventricular outflow tract associated with disseminated intravascular coagulopathy. Kyobu Geka 1999; 52: 401-4. 414 H.-Y. Cho, M.-K. Kim, S.-J. Cho, et al.