Arch GynecolObstet(2@8) 277:179-182 DOI I 0.I 007/s00404-007-0450I
CASE REPORT
Low-grade endometrial stromal sarcoma with retroperitoneal
metastases: an unusualcasereport Kadir Guzehneric . Bahar Ergen . Zehra Meltem Pirimoglu. Melin Ozgun Gecer . Orhan Unal . Cem Turan
Received: 13 May 2007 I Accepted:15 August 2007 / Publishedonline: 7 September2007 @ Springer-Verlag?-N7
Abstract Background Endometrial stromal sarcoma @SS) is an uncommon malign neoplasm, and its occurrence outside the uterus is extremely rare in the absence of metastasis or €xtension of a primary uterine neoplasm. When arising in the pelvis or abdominal cavity, ESS is associatedwith uterine adnexa or serosal surface of various organs. Case We present the case of a 46-year-old woman with lower abdominal pain and regular menstruation who underwent laparotomy after a diagnosisof pelvic mass mimicking a right adnexial tumor. Exploration of the pelvis revealed a retroperitoneal mass of 15 cm in diameter in the right illiac fossa without accompanying pelvic or paraaortic lymphadenopathy. Uterus and ovaries were bilaterally normal in size. The pathology showed low-grade ESS of the uterus with direct spreadto retroperitoneum without serosal metastases. Conclusion This case shows that desprte its well-known good prognostic nature, low-grade ESS may behave as an aggressivemalignancy.
K. Guzelrneric.B. Ergen.Z. M. Pirinoglu.O. Unal . C. Turan Deparment of Obstetricsand Gynecology, Dr. Lud Kirdar Kartal Educationand ResearchHospital, Istanbul,Turkey M. O. Gecer Departmentof Pathology, Dr. Lud Kirdar Kanal Educationand ResearchHospital, Istanbul, Turkey K. Guzelmeric (E) 34 Ada Yesim Sitesi Blok I2Due 8 Atasehir, Kadikoy, 34758Istanbul, Turkey e-mail:
[email protected]
Keywords Endometrialstromalsarcoma. Retroperitoneum' Low-grade
Introduction Stromal sarcomaof uterusis a rare neoplasm,which is classically divided into low-grade endometrial stromal sarcoma @SS) and high-grade ESS. The former is a malignant tumor with endometrial sfromal differentiation and characteized by a cellular proliferation of spindle cells usually with mild nuclear atypia, associated with numerous small arterioles, mimicking spiral arterioles of the endometrium. The latter consistsofneoplastic cells with obvious anaplastic features [1, 2]. This classificationdependson the basis of mitotic activity. According to Evans [3] the clinical relevance of dividing ESS intoflow-grade and high-grade tumors has become inconsistent. While high-grade undiferentiated stromal sarcoma tends to have poor prognosis, low-gradeESS usually have excellentshort-termprognosis. Low-grade ESS microscopicallyhas fewer than 10 mitosis per 10 high-power fields (HPF) and a clinically protracted course. High-grade ESS often completely lacks stromal differentiation and exhibits more than 10 mitosis per l0 IIPF [2]. Clinically it has a more aggressiveclinical course and a poorer prognosisthan low-gradeESS [1, 2]. Low-gradeESS nearly always begins in the endometrium or myometrium, although unusual casesarising from endometriosis, particularly in the ovary has been recorded [4]. Almost always, low-grade ESS is found on a hysterectomy specimen without any suspicion of diseasebefore surgery. It spreadsto pelvis, lower genital tract, and lungs and rarely to other sites [5]. We present an unusual case of low-grade ESS with retoperitoneal metastaseswithout any serosal invasionof uterus,oviductsor lymph node metastases. -0 Springer
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Case report
Pathology
A 46-year-old gravida 11, para 8, perimenopausalwoman was admitted to the hospital with lower abdominal pain and abdominalfullness. Her menstrualcycle was regular. Physical examination revealed a tender fixed palpable solid mass in the iliac fossa. Transabdominal and transvaginal ultrasoundexaminationshoweda 115 mmx 84 mm, a solid and cystic heterogeneousmass that could not be distinguished from right ovary adjacent to uterus (Fig' l). The uterus was mildly enlarged. Endometrial thickness was 6 mm and was regular in structure.The patient had no history of antecedentlaparotomy. At admission her serum CA125, CAI}-9, CEA, Alfa-fetoprotein, LDH and beta-hCG levels were normal. At laparotomy, uterus was mildly enlarged and ovaries bilaterally were nortnal. Exploration of the pelvis revealed a retroperitoneal mass 15 cm in diameter in the right iliac fossa. There was no infiltration on pelvic and abdominal organs. The retroperitoneal space was entered easily. The mass was fragile with prominent neo-vascularization,so mild bleeding occurred during the process.After the excision of the mass,anotherbut smaller and more fibrous mass was found below the original mass in the obturator fossa. The secondsmaller masswas excisedas well. After frozen sectionsof the tumors revealed well-differentiated sarcoma, a total hysterectomy, bilateral salpingo-oophorectomy, pelvic and paraaortic lymphadenectomy were performed' There was no macroscopicresidual diseaseat the end of the procedure.The patient was commencedon medroxyprogesterone acetate. At end of the 12 months postoperatively, therehas beenno recurent disease.
The retroperitoneal mass had hypercellular zones with foci of cystic and hyaline degeneration. Diffirse necrotic and hemorrhagic foci were found. The tumor cells had uniform round or oval nuclei and scanty cytoplasm with ill-defined cell boarders(Fig.2). The small blood vesselswere distributed uniformly among the cells (Fig. 3). Pleomorphic or enlarged nuclei were rare. Mitotic index was two per 10 high-power fields. Myometrium showedalso similar pathological featuresin a few local areas(Fig. a). No lymph vascular spaceinvasion was found in the uterine specimen.
Fig. 1 lJinasonographicappearanceof the tumor in the peivis of the patient
Q Springer
Fig. 2 The tumor cells have uniform round or oval nuclei and scanty cltoplasm with ill-defined cell borders
Fig.3 The numeroussmall spiral vesselsdistributedrelatively uniformjy amongthe tumorcells
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triosis [4, 8]. Low-grade ESS has been found to be , extendedbeyond the uterusincluding ovary, fallopian tpbe, cervix, vagina, vulva pelvic cavity and abdominal cavity at the tirne of diagnosis n 40Voof cases[5, 9-12].In some cases,the lesions have been associatedwith endomptriosis [E]. Casesof extrauterineESS in the pelvis or abdominal cavity are associated with the serous surface of organs. In this oase, although the primary tumor originated from uterus, no metastasiswas found on seroussurfacesin the abdominal cavity in the presence of retroperitoneal tumor metastasis.Since the patient did not complain of any abnormal uterine bleeding and physical examination showed no uterine enlargement, the complex solid mass may more likely to be adnexial pathology. However, the pathological investigation revealed the presenceof a uterine tumor. Its Fig.4 Transitionzonebetweenstromaltumorandmyometrium occurrence as an incidental finding in uteri removed for otherreasons,and dicroscopic sizeof the tumor in myometrium is usually seenin adenorhyosiscasesmimicking ESS While'endometrium was showing irregular proliferation, both ovaries and fallopian tubes were regular and norrnal [9]. In this case,the apparenttransition zone betweenstromal tumor and rnyometrium was microscopically deterhistologically.Lymph nodgsshowedno evidenceof Eetasrnined (Fig. a), tases. The tendency for local lymphatic invasion in low-grade Immunohistochemicalstudiesshowedthat the neoplastic cells were immunofeactive to CDl0, CD68, progesteron ESS is well established[5]. It seemglogical that the tumor would spread to local regional lymph nodes as illustrated rep€ptor,estrogenreceptorand vimentin.l
