| Abstract|| |
Primary leiomyosarcoma (PLMS) of the ovary is extremely rare tumors comprising 1% of ovarian tumors. About 3% of all ovarian malignancies are primary ovarian sarcomas. Only 72 cases have been reported till date. A 57-year-old postmenopausal female presented with abdominal pain for the last 6 months. Ultrasonography and MRI revealed a heterogeneously enhancing solid lobulated mass in the left adnexa abutting the fundus of the uterus and bowel loops. The endometrial cavity was normal. Ovarian markers CA 125, CEA, CA 19.9, and all hematological parameters were within normal limits. LDH was near normal (284 IU/ml). The specimen was sent for frozen section and a diagnosis of malignant spindle cell lesion of ovary was rendered. Histopathology of the ovarian mass revealed intersecting fascicles of tumor cells consisting of ovoid to spindle-shaped cells having a moderate amount of cytoplasm. Bizarre and atypical cells were seen singly dispersed and in small aggregates along with the brisk mitotic activity. Focal areas of necrosis and hemorrhage were also noted. Immunohistochemistry showed strong positivity for smooth muscle actin and Caldesmon while focal positivity for Desmin and Epithelial Membrane Antigen (EMA) was noted. The lesion was negative for Inhibin, Calretinin, and CD 117 and S100. The final diagnosis of primary ovarian Leiomyosarcoma was given based on histopathology and Immunohistochemistry. PLMS of the ovary are rare incidental findings in postmenopausal women. These are highly malignant tumors and carry a poor prognosis. Hence, early diagnosis and surgical treatment with cytoreduction improve patient survival.
Keywords: Caldesmon, leiomyosarcoma, ovarian, primary, smooth muscle actin, surgery
|How to cite this article:|
Raychaudhuri S, Sidam D, Jain M, Chawla R, Pujani M, Wadhwa R. Primary leiomyosarcoma of ovary: A rare malignancy as an incidental finding. Indian J Pathol Microbiol 2022;65:938-41
|How to cite this URL:|
Raychaudhuri S, Sidam D, Jain M, Chawla R, Pujani M, Wadhwa R. Primary leiomyosarcoma of ovary: A rare malignancy as an incidental finding. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Nov 30];65:938-41. Available from: https://www.ijpmonline.org/text.asp?2022/65/4/938/359366
| Introduction|| |
Primary ovarian sarcomas are very rare and comprise only about 3% of all ovarian malignancies. Leiomyosarcoma primarily arising in the ovary is an extremely rare subtype of ovarian sarcomas.
The ratio of sarcoma to carcinoma in ovaries is found to be1:40. Primary ovarian leiomyosarcomas constitute only 1% of ovarian tumors.
Only 72 cases of the disease have been reported till date.
The older age group of more than 60 years are most commonly affected and are diagnosed at an advanced stage with most deaths occurring in the first year of diagnosis.
These are highly malignant and metastasizes mainly to the lungs and liver and 5-year survival is 20 to 30%.
The treatment is of choice is surgery. The role of radiotherapy and adjuvant chemotherapy cannot be ascertained due to the rarity of cases. We discuss this rare malignancy by describing a case report.
| Case Report|| |
A 57-year-old post-menopausal lady presented to the gynae OPD with complaints of pain abdomensince6 months. The general physical examination, biochemical and hematological examinations were normal.
CEMRI (Contrast-Enhanced Magnetic Resonance Imaging) of the pelvis showed a lobulated heterogeneously enhancing, predominantly solid mass lesion in the left adnexa with few hemorrhagic foci, possibly an ovarian mass. Medially the lesion was seen abutting the fundus of the uterus with indistinct fat planes and also the bowel loops with no evidence of infiltration. The endometrial cavity was normal. Bilateral ovaries were not seen separately [Figure 1]a and [Figure 1]b.
|Figure 1: (a) and (b). CEMRI shows a lobulated heterogeneously enhancing predominantly solid mass lesion in the left adnexa medially abutting the fundus of the uterus|
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The ovarian tumor markers like CEA, CA19.9, and CA 125 were normal except LDH which was near normal, 284 IU/ml.
Exploratory laparotomy showed a mass located between the uterine fundus and the pouch of Douglas. The mass originating from the left ovary was removed and sent for a frozen section [Figure 2]a and [Figure 2]b
|Figure 2: (a) Gross-Outer surface is bosselated. (b) Cut surface shows grey white fleshy lobulated appearance|
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Since the frozen section examination suggested a malignant spindle cell lesion, staging surgery (type 1 hysterectomy, bilateral salpingo-oophorectomy, infracolic omentectomy, peritoneal biopsy, and cytology, bilateral paraaortic-pelvic lymphadenectomy, left external iliac, and bladder peritoneal biopsy) was performed. Surgical specimens were sent for histopathological examination.
Diagnosis of malignant spindle cell lesion, possibly primary ovarian leiomyosarcoma was proposed and Immunohistochemistry was advised [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d.
|Figure 3: (a). Intersecting fascicles and bundles of ovoid to spindle cells. 20×; H and E. (b). Bizarre atypical cells having brisk mitotic activity. 20×; H and E. (c). Areas of necrosis. 20×: H and E. (d). Intersecting fascicles with area of pleomorphism. 20×: H and E|
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The tumor was restricted within the ovarian capsule with no extension into the serosal surface.
Immunohistochemistry revealed strong cytoplasmic diffuse positivity for smooth muscle actin and caldesmon and focal cytoplasmic positivity for Desmin while the tumor was negative for Inhibin, Calretinin, CD 10 and CD 117, S-100, and cytokeratin confirming the diagnosis of leiomyosarcoma of ovary [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d.
|Figure 4: (a, b) IHC showing strong diffuse positivity for smooth muscle actin and Caldesmon. (c) IHC showing focal positivity for Desmin. (d) IHC showing negativity for calretinin|
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The sections from the specimens labeled as hysterectomy with one-sided adnexa, infracolic, omentum, bilateral peritoneal biopsy, bilateral pelvic lymph nodes, and external iliac lymph nodes and bladder peritoneal biopsy were all free of tumor.
| Discussion|| |
Pure primary leiomyosarcoma (PLMS) of the ovary is very rare (<1%). The most common sarcomas of the ovary are fibrosarcoma, endometrial stromal sarcomas, and rhabdomyosarcoma.
The origin and genetics of ovarian sarcomas are not clear. It is suggested that it arises from the smooth muscle components of the ovary. Ovarian leiomyoma can undergo malignant transformation or migration of uterine leiomyoma can also occur, although these are very rare.
It can arise from the smooth muscles around the corpus luteum, ovarian follicles, and ligaments, blood vessels, remnants of Wolfﬁan ducts, and ovarian mesenchymal totipotent cells. It can also arise from differentiated teratoma cells or smooth muscle cells of the uterus that have migrated.
Most cases of leiomyosarcoma present with vague signs and symptoms such as abdominal bloating, pelvic pain, and discomfort with pressure symptoms on the bowel and bladder.,
The present case also had abdominal pain.
These tumors usually occur in postmenopausal women, but sometimes younger women may be affected. The age range for ovarian leiomyosarcoma is 12 to 84 years with a mean of 52.6 years.
The typical presentation is a large unilateral mass predominantly affecting postmenopausal women.
The present case showed a left-sided solid abdominal mass, which on cut section appeared grey white, irregular, lobulated with focal areas of hemorrhage.
Histology shows fascicles of spindle cells arranged in intersecting bundles having cells that are fusiform and pleomorphic with elongated nuclei and prominent nucleoli and eosinophilic cytoplasm., Nuclei have blunted or truncated ends and have dense cytoplasm.
The criterion for diagnosis is hypercellularity, nuclear atypia, pleomorphism, coagulative necrosis and high mitotic activity (>5 mitoses/10 hpf)
This case also showed fusiform cells with elongated nuclei having moderate eosinophilic cytoplasm with bizarre atypical cells having high and atypical mitosis (6 to 7 mitosis/10 hpf) with focal areas of necrosis and haemorrhage.
Differential diagnosis of LMS includes leiomyoma, fibrothecoma, fibrosarcoma, endometrial stromal sarcoma, spindle cell carcinoma, and Gastro-Intestinal Stromal Tumour.
Leiomyosarcomas are immunoreactive for SMA, Desmin, Vimentin, and Caldesmon but negative for S-100 and Cytokeratins.
Leiomyomas are strongly positive for SMA but lack cellular and nuclear pleomorphism and mitotic activity.
Fibrothecoma is positive for CD 10 and Inhibin.
Fibrosarcomas are negative for Desmin and show an orderly arrangement of spindle cells with no pleomorphism.
Endometrial stromal sarcomas of ovary are negative for Inhibin but are positive for CD 10.
Gastrointestinal stromal tumors express CD 117 and DOG 1.
The present case showed strong cytoplasmic positivity for SMA and Caldesmon but focal cytoplasmic positivity for Desmin and EMA.
Calretinin, Inhibin, and CD 10 were negative ruling out germ cell malignancies.
C kit was negative ruling out the gastrointestinal stromal tumor.
Leiomyosarcomas are diagnosed at advanced stages when they measure more than 10 cm and have aggressive behavior with a poor prognosis. Most patients die within the first year of diagnosis but in our case, the patient was lost to follow up.
Early relapses are common to the abdomen and pelvis but less frequent to lungs, liver, bone, and mediastinum, the cerebrum.
Post-operative radiology controls the local disease while post-operative chemotherapy prevents distant metastasis.
For in operable tumors with a poor prognosis, adjuvant therapies may be more useful.
Less than 100 cases are reported in the literature to date, hence more extensive studies are needed to know the exact nature, behavior, and treatment modalities of LMS.
| Conclusion|| |
Primary LMS of the ovary is a rare mesenchymal tumor with poor outcomes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of Interest
There are no conflicts of interest.
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