|Year : 2016 | Volume
| Issue : 1 | Page : 109-111
Hyaline vascular variant of low-grade endometrial stromal sarcoma masquerading as ovarian tumor
Kavita Mardi, Deepak Vedant
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Web Publication||2-Jun-2016|
Set No. 14, Type VI, IAS Colony, Meheli, Shimla, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Endometrial stromal sarcomas (ESSs) are uncommon tumors of the uterus, and the cytologic features have only been reported in a number of case reports that mostly discuss the features of higher grade undifferentiated sarcomas. This article discusses the cytologic features of a unique case of hyaline vascular variant of low-grade ESSs (LGESS), which masqueraded as a ovarian tumor due to its massive extension into broad ligament. Touch imprint smears revealed bland cells occurring singly and small clusters, scant cytoplasm, small round or oval nuclei, fine chromatin pattern, and occasional small prominent nucleoli. Frequent metachromatic hyaline globules and plaques were seen between the tumor cells in all the smears. Histological examination of the tumor showed LGESS with extensive stromal and perivascular hyalinization.
Keywords: Endometrial stromal sarcoma, Fine needle aspiration cytology, hyaline vascular variant, low-grade
|How to cite this article:|
Mardi K, Vedant D. Hyaline vascular variant of low-grade endometrial stromal sarcoma masquerading as ovarian tumor. Arch Med Health Sci 2016;4:109-11
|How to cite this URL:|
Mardi K, Vedant D. Hyaline vascular variant of low-grade endometrial stromal sarcoma masquerading as ovarian tumor. Arch Med Health Sci [serial online] 2016 [cited 2020 Jul 13];4:109-11. Available from: http://www.amhsjournal.org/text.asp?2016/4/1/109/183361
| Introduction|| |
Some low-grade endometrial stromal sarcomas (LGESSs) show hyalinized foci with the formation of collagen in thick bundles and extensive perivascular hyalinization. Such tumors are called “hyaline vascular type” of LGESS. Such variants are extremely rare. The cytologic features of these tumors have been reported in only a small number of case reports and most reports describe the features of high-grade undifferentiated sarcomas. We report cytological and histopathological findings in a case of hyaline vascular variant of LGESS masquerading as an ovarian tumor in a 45-year-old female. Touch imprint cytology of the tumor revealed the characteristic findings of the tumor along with abundant metachromatic hyaline globules and plaques. Unusual clinical presentation and unique cytological as well as histological findings made us report this case.
| Case Report|| |
A 45-year-old female presented with dull aching pain in the lower abdomen for the past 14 years, and gradually progressive abdominal distension for about 3 months. Abdominal examination a mass of about 30 weeks size was palpable. The mass was nontender, mobile from side to side with regular margins. Computed tomography scan revealed a heterogenous solid cystic intra-abdominal and pelvic mass. Radiological diagnosis of malignant solid cystic ovarian tumor was made. Per operatively, the surgeons found a unilocular ovarian tumor with a solid area measuring 4 cm × 5 cm. In addition, they found a fibroid measuring 5 cm × 5 cm on posterior surface of uterus. Total abdominal hysterectomy with bilateral salphingo-oophorectomy along with the ovarian tumor was done. A volume of 50 ml clear fluid was present in peritoneal cavity. Adhesiolysis of caecum and appendix from the posterior surface of ovarian tumor was also done. Gross specimen along with touch imprint smear from the ovarian tumor was sent to us for examination.
Microscopic examination of the Giemsa-stained imprint smears revealed bland cells occurring singly and in small clusters, with scant cytoplasm, small round or oval nuclei, having fine chromatin pattern, and occasional small prominent nucleoli. Frequent metachromatic hyaline globules and plaques were seen between the tumor cells in all the smears [Figure 1].
|Figure 1: Touch imprint smears revealing plaques and globules of metachromatic material between the bland tumor cells (Giemsa, ×40)|
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On gross examination of the specimen, we found a fleshy grey white growth in the myometrium measuring 10 cm × 7 cm × 5 cm infiltrating transmurally and extending and infiltrating the layers of broad ligament [Figure 2]. Cut surface of the growth was gray white, friable with areas of hemorrhage and necrosis. Same side Fallopian tube More Details and ovary were stretched and pushed to one side over the cyst. Microscopic examination of the myometrial tumor showed sheets of uniform cells, invading transmurally. These cells were showing round to ovoid nuclei with fine granular chromatin, inconspicuous nucleoli and scant cytoplasm with indistinct cell borders. Throughout the tumor, there were round to elongated eosinophilic, hyaline areas as well as marked perivascular hyaline change [Figure 3]. Only occasional mitotic figures were seen. In addition, there were marked areas of hemorrhage and necrosis. The tumor was also infiltrating between layers of the same side broad ligament. Both side ovaries were within normal histological limits. A diagnosis of hyaline vascular variant of LGESS was made.
|Figure 2: Gross specimen showing growth in the endometrium as well as in broad ligament|
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|Figure 3: Photomicrograph revealing low-grade endometrial stromal sarcomas with extensive stromal and perivascular hyalinization (H and E, ×40)|
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| Discussion|| |
Low-grade ESSs are rare malignant tumors that comprise only about 0.2% of all female genital tract malignancies. These tumors histologically and immunophenotypically resemble the normal endometrial stroma. The most recent WHO classification for tumors of the uterus classifies the tumors based primarily on tumor margin status and cytologic features. Low-grade ESSs are clinically indolent malignancies with minimal cytologic atypia and proliferative activity.
Compared with other uterine malignancies, they occur at an earlier age (42-58 years), and about 10-25% of the patients are premenopausal. The tumors have an indolent growth with a tendency for late recurrence. Metastases are rarely detected before the diagnosis of the primary lesion. The present tumor showed unusual presentation with extensive involvement of parametrium/broad ligament, because of which radiologically this tumor was diagnosed as a malignant solid cystic ovarian tumor.
The histologic features include cords of tumor cells infiltrating between smooth muscle and within lymphatic spaces. The neoplastic stromal cells resemble those of the stromal cells of the proliferative endometrium, are monotonous in appearance, and have relatively uniform size and shape. Mitotic activity is usually low (<10/10 high power fields). Proliferating small vessels resembling the endometrial spiral arterioles are characteristic, and tumors can have bands of hyaline connective tissue separating islands and clusters of bland neoplastic stromal cells.
Some LGESS show extensive perivascular hyalinization. Such tumors are called “hyaline vascular type” of LGESS. As hyalinization is associated with endometrial stromal nodule and LGESS, but not with high-grade ESS, presence of hyalinization is suggestive of low-grade tumors.
By fine needle aspiration (FNA), the cytologic features better recapitulate the histologic features, and a vascular and hyalinized stroma are often seen. The previously described cytologic findings of LGESS such as bland cells occurring singly and small clusters, scant cytoplasm, small round or oval nuclei, fine chromatin pattern, and occasional small prominent nucleoli ,,, were observed in our case also. Most cytologic samples of LGESS will include a combination of single cells and stromal fragments with interspersed blood vessels.
Metachromatic hyaline stromal matrix and intervening vessels, if present together, may assist with the diagnosis on an FNA specimen but cannot usually be identified with gynecologic preparations. “Comet cells” which are spindle-shaped cells with eccentric nuclei and cytoplasm tapering away from the nucleus are seen in some of the cases.
In conclusion, because of the bland morphologic features of these tumors, familiarity with their clinical and cytologic features may allow one to correctly diagnose or, at least, suggest the diagnosis of these lesions.
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[Figure 1], [Figure 2], [Figure 3]