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OJHAS Vol. 8, Issue 3: (2009
Jul-Sep) |
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Primary Leiomyosarcoma of Ovary |
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Rashmi Kaul, Senior Resident,
Jaishree Sharma, Ex-Professor & Head
Department of Pathology, Indira Gandhi Medical College, Shimla - 171001, Himachal Pradesh
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Address For Correspondence |
Fire Officers Building,
Stokes Place,
Shimla (H.P) 171002
India
E-mail:
shivanshraina@yahoo.co.in |
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Kaul R, Sharma J. Primary Leiomyosarcoma of Ovary. Online J Health Allied Scs.
2009;8(3):16 |
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Submitted: Jul 20, 2009; Accepted:
Aug 18, 2009 Published: Nov 15, 2009 |
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| Abstract: |
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Primary ovarian leiomyosarcomas is a rare neoplasm
which comprises less than 3% of ovarian tumors. Their origin, etiology, histologic features, clinical behavior, and
optimal treatment are still obscure. We report a case of leiomyosarcoma of ovary, diagnosed on histopathology in a 60
year old female and discuss the literature
Key Words: Ovary, Leiomyosarcoma, Ovarian neoplasms
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Primary ovarian sarcomas are rare gynecological
tumors accounting for less than 3% of all ovarian tumors.[1,2] These malignant smooth muscle tumors have an uncertain
origin, since smooth muscle is not present in the ovary. Behavior is aggressive with a poor prognosis particularly in
the postmenopausal woman, where these neoplasms usually occur. Primary ovarian sarcomas are usually diagnosed at an
advanced stage and may present with abdominal pain and a mass. These neoplasms are rare with less than 50 cases
in the literature.[1] Because of its extreme rarity; we present this case of ovarian neoplasm in a postmenopausal
woman. Literature of primary leiomyosarcoma of the ovary is reviewed.
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A 60 year old female, para six, postmenopausal
for 20 years, was admitted in the gynecology department of our hospital with complaints of having lower abdominal
mass for last five months. She was found anemic on general physical examination. On per vaginum examination a mass
was felt in right fornix of variegated consistency with restricted mobility and was non tender. On per rectal
examination, mass was felt from anterior rectal wall but rectal mucosa was free. Her hemoglobin was 7.2 gm%.
Hematological and biochemical parameters including tumor marker CA-125 were normal. Cervical PAP smear was also
normal. Transabdominal sonography confirmed the presence of a 12.8x10.3 cm pelvic mass arising from right adnexa
with cystic and solid areas, separated by septations. There was no
evidence of metastasis or free fluid in the pelvis. At laparotomy, right ovary was replaced by a friable growth of 25×20 cm size with capsule ruptured, solid
areas and papillary excrescences present. Tumor was involving 20cm of gut loop in ileal
portion. Uterus, left fallopian tube and left ovary were grossly
normal.
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Figure 1: Photomicrograph showing pleomorphic tumor cells, high mitotic activity and tumor giant cells along with a focus of necrosis. (400x, H&E.) |
Total abdominal hysterectomy with bilateral salpingo-oophorectomy
along with ileo-ileal anastmosis was performed. Peritoneal washings with omental biopsy were taken. Microscopic
examination demonstrated ovarian stroma replaced by highly vascular tumor, pleomorphic spindle shaped cells,
increased mitotic activity (greater than 10 per 10 high power fields), tumor giant cells and necrosis.(Fig-1)
The pleomorphic cells had abundant eosinophilic cytoplasm with vesicular nuclei and prominent nucleoli.
Van Gieson’s and Masson’s trichome stains helped to confirm the smooth muscle origin. Microscopic examination
of left ovary revealed normal ovarian stroma along with foci of whorled bundles of benign smooth muscle cells,
depicting features of leiomyoma. No leiomyoma were detected in sections taken from uterus and tubes.[2] Peritoneal
washing, omental biopsy and gastrointestinal tract mucosa was normal on histopathology.
The most common primary ovarian sarcomas
are fibrosarcomas, endometrial stromal sarcomas, and rhabdomyosarcoma.[3,4] Primary ovarian leiomyosarcomas
usually occur in postmenopausal women although there have been a few reported cases occurring in younger women.[1,3]
They typically present as a solitary, lobular, soft fleshy solid mass with hemorrhage and cystic degeneration. Most
are unilateral lesions greater than one cm in diameter. Pathogenesis is uncertain with many theories including
malignant degeneration of an ovarian leiomyoma, or of the smooth muscle present in the wall of the blood vessels in
the cortical stroma and corpus luteum, muscular attachments of the ovarian ligament, wolfian duct remnants, or
totipotential ovarian mesenchyme, or arising in a teratoma.[1] Treatment of choice is radical surgery followed by
adjuvant chemotherapy or radiotherapy.[4]
- Bouie SM, Cracchiolo B, Heller D. Epithelioid leiomyosarcoma of the ovary.
Gynecologic Oncology 2005;97:697–9.
- Dixit S, Singhal S, Baboo H, Vyas RK, Neema JP, Murthy R et al.
Leiomyosarcoma of the ovary.
J Postgrad Med 1993;39:151–3
- Friedman HD, Mazur M. Primary ovarian leiomyosarcoma. An
immunohistochemical and ultrastructural study.
Arch Pathol Lab Med 1991;115:941–5.
- Kurian RR, Preethi J, Remadevi AV. Leiomyosarcoma of ovary- a case report.
Indian J Pathol Microbiol 2005;48:19-20
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