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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 2  |  Page : 217-218
Primary pleomorphic sarcoma of the ovary with rhabdomyosarcomatous differentiation


Department of Pathology, Bankura Sammilani Medical College, Kolkata, India

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   Abstract 

A 49-year-old patient presented with a right ovarian mass, which, on microscopy, showed to consist of haphazardly oriented large pleomorphic cells with abundant cytoplasm. Periodic acid Schiff stain was positive but negative with diastase digestion. Immunohistochemical staining with Desmin showed intense cytoplasmic positivity in almost all the cells. Cytokeratin, epithelial membrane antigen, smooth muscle actin, HMB-45, S-100 and neurone-specific enolase were negative. Immunohistochemical staining with Myogenin showed intense nuclear positivity. There was no other primary tumor on extensive search. A diagnosis of primary sarcoma of the ovary with rhabdomyosarcomatous differentiation was made. The incidence of similar tumors of the ovary are low and therefore little data are available on this uniformly lethal tumor. Thus, such cases need to be reported to pool experience so that the tumor can be diagnosed early.

Keywords: Desmin, myogenin, ovary, rhabdomyosarcomatous, sarcoma

How to cite this article:
Mukherjee S, Sen S, Biswas P, Choudhuri M. Primary pleomorphic sarcoma of the ovary with rhabdomyosarcomatous differentiation. Indian J Pathol Microbiol 2009;52:217-8

How to cite this URL:
Mukherjee S, Sen S, Biswas P, Choudhuri M. Primary pleomorphic sarcoma of the ovary with rhabdomyosarcomatous differentiation. Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Sep 19];52:217-8. Available from: http://www.ijpmonline.org/text.asp?2009/52/2/217/48923



   Introduction Top


Primary sarcomas represent only 2-3% of all female genital tract malignancies. Only 10% occur outside the uterus (vulva, vagina, ovaries,  Fallopian tube More Detailss and uterine ligaments). [1] Mesenchymal tumors are present in the ovary only occasionally. Pre-operative diagnosis is unlikely and surgical exploration is mandatory. Because these tumors are highly uncommon, scant clinical or molecular studies are available. [2] Hence, similar cases need to be reported as the prognosis is bad and pooling of experience could help in early diagnosis.


   Case Report Top


A 49-year-old female patient was admitted to our hospital with complaints of pain in the lower abdomen. Ultrasound examination revealed a solid mass in the right ovary of size 7cm × 6cm × 3cm. The left ovary uterus and cervix were unremarkable.

Pre-operative routine tests were normal except for moderate anemia. Laparotomy was performed. The right ovary was involved by the tumor. The uterus and the left appendages appeared to be healthy. Omental nodules were present, which were biopsied. The patient had an uneventful recovery.

Morphological findings

Grossly, the tumor was ovoid in shape and measured 7.5cm × 6cm × 3cm, was greyish white in color and the peritoneal surface was smooth. The cut section was solid and fleshy with focal areas of necrosis and hemorrhage. The gross features were suggestive of malignancy.

The tumor showed absence of a surface layer of cuboidal cells. The cells were loosely arranged, haphazardly oriented, large, round, pleomorphic with hyperchromatic nuclei and abundant eosinophilic cytoplasm. Some cells had a peripherally located nucleus, which was vesicular with a prominent nucleolus [Figure 1]. The following three differential diagnoses were considered:

  1. Primary sarcoma of the ovary.
  2. Metastasis from poorly differentiated carcinoma.
  3. Primary malignant melanoma.


Periodic acid Schiff stain was performed, which was positive, but negative with diastase digestion.

No striations could be identified. Immunohistochemistry revealed intense cytoplasmic positivity for desmin in almost all the cells. Cytokeratin, epithelial membrane antigen, HMB-45, neurone-specific enolase, smooth muscle actin and S-100 were negative. Thus, metastasis from the epithelial malignancy and malignant melanoma were ruled out. The possibilities of malignant peripheral nerve sheath tumor and leiomyosarcoma were also ruled out. The strongest possibilities were either a pleomorphic rhabdomyosarcoma or a primary malignant fibrous histiocytoma. Myogenin stain was performed and showed strong nuclear positivity [Figure 2]. Hence, a diagnosis of primary sarcoma of the ovary with rhabdomyosarcomatous differentiation was proved.

Further sections were taken to determine whether the sarcoma was primary to the ovary or a component of mixed tumor or teratoma. Epithelial elements, cartilage or bone could not be detected. It was clear that the tumor was neither a teratoma nor a mixed tumor.

The sections from the omental nodules showed identical histological features.

The patient underwent chest X-ray and a computed tomographic scan of the whole abdomen was performed after thorough clinical examination to ensure that there was no primary tumor elsewhere.

The patient was put on chemotherapy but was lost to follow-up.


   Discussion Top


Pure rhabdomyosarcomas of the ovary are extremely rare and are lethal primary neoplasms. This report describes the light microscopic and immunohistochemical findings of the case. Very few reports of primary rhabdomyosarcomas of the ovary have been reported. [3],[4],[5] Most reported cases were of embryonal rhabdomyosarcoma and in children.

The age of patients at diagnosis ranged between 13 months and 86 years, with 60% of women aged 40 years and older. About 50% of the cases were diagnosed with disease beyond the ovary and survival ranged between 18 days and 15 months after diagnosis. [3] In this case, the patient was 49 years of age but unfortunately the period of survival or results of treatment could not be determined as the patient was lost to follow-up.

The microscopic features of ovarian sarcomas resemble their counterparts in other sites. [6] The most helpful light microscopic feature that suggests this diagnosis is the presence of large bizarre tumor cells with a deeply eosinophilic cytoplasm. Rare lesions have cells with a rhabdoid morphology characterized by the presence of a peripherally located vesicular nucleus with a prominent nucleolus and an intracytoplasmic eosinophilic hyaline inclusion. Cells with cross striations commonly found in embryonal rhabdomyosarcoma are rare in adult pleomorphic rhabdomyosarcoma. [7] Our case had focal areas of cells with an eccentric vesicular nucleus and a prominent nucleolus.

Rhabdomyoblasts in the ovary may arise from either the uncommitted stromal fibroblasts or from fibroblasts of the endometriotic stroma. [3] If it is supposed that the tumor is teratomatous in origin and arises from a totipotent cell, it is difficult to understand why only the myogenic cells are represented and why the epithelial cells are absent. Although the tumor in this case arose from the ovary where totipotent cells are known to exist, the incidence of such tumors in the ovary is small and similar tumors occur more commonly in organs where totipotent cells are not present. It seems that for the present no satisfactory explanation can be given about the origin of these tumors.

 
   References Top

1.Leppien G. Non uterine gynaecologic sarcoma. Arch Gynecol Obstet 1987;241:25-32.  Back to cited text no. 1  [PUBMED]  
2.Jordens N, Pauw AM, Vergote I, Amant F. Molecular markers of Mesenchymal tumors. CME J Gynecol Tumors 2006;11:73-7.  Back to cited text no. 2    
3.Guerard MJ, Arguelles MA, Ferenczy A. Rhabdomyosarcoma of the ovary: Ultrastructural study of a case and review of literature. Gynecol Oncol 1983;15:325-39.  Back to cited text no. 3    
4.Cribbs RK, Shehata BM, Ricketts RR. Primary ovarian rhabdomyosarcoma in children. Pediatr Surg Int 2008;24:593-5.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Paler RJ Jr, Felix JC. Desmin, myoglobin and muscle specific actin immunohistochemical staining in a case of embryonal rhabdomyosarcoma of the ovary. Appl Immunohistochem Mole Morphol 1999;7:237.  Back to cited text no. 5    
6.Clement PB, Young RH, Scully RE. Miscellaneous primary tumors, secondary and non-neoplastic lesions of the ovary. In: Mills SE, editor. Sternberg's diagnostic surgical pathology. 4 th ed. Lippincott Williams and Wilkins; 2004. p. 2618.  Back to cited text no. 6    
7.Weiss SW, Goldblum JR, editors. In: Enzinger and Weiss' soft tissue tumors. 4 th ed. Mosby; 2001. p. 814-7.  Back to cited text no. 7    

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Correspondence Address:
Sumana Mukherjee
BH-62, Sector-2, Salt Lake, Kolkata - 700 091
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.48923

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    Figures

  [Figure 1], [Figure 2]

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    Abstract
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