Leiomyosarcoma of the ovary.S Dixit, S Singhal, HA Baboo, RK Vyas, JP Neema, R Murthy, U Sooryanaraya
Dept of Radiation Oncology, Gujarat cancer and Research Institute, New Civil Hospital Compound, Asarwa, Ahmedabad.
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 0008051647
Source of Support: None, Conflict of Interest: None
A diagnosis of leiomyosarcoma of ovary was made in a 60 year old female presenting with generalised weakness and abdominal lump. On clinical examination, a hard, big mass with some cystic areas was found occupying the pelvic cavity. Chest X-ray revealed presence of metastases. Deranged renal function and structure due to extrinsic pressure were evident on pyelography and USG. USG also suggested the ovarian origin of the mass. Fine needle aspiration biopsy was suggestive of leiomyosarcoma. Laparotomy was carried out for excision of tumor along with bilateral salpingo-oophorectomy and hysterectomy. Post-operatively renal functions normalized. A course of radiotherapy was given. At 6 months' follow-up, abdomino-pelvic sonography was normal but lung metastases were found to be enlarged. The patient was asked to follow up for chemotherapy but did not come. She died 18 months after treatment, as revealed through correspondence.
Keywords: Case Report, Combined Modality Therapy, Fatal Outcome, Female, Human, Hysterectomy, Leiomyosarcoma, pathology,secondary,therapy,Lung Neoplasms, pathology,secondary,therapy,Middle Age, Ovarian Neoplasms, pathology,therapy,Ovariectomy,
Primary soft tissue sarcomas of the ovary are rare tumours. They are often confused with undifferentiated carcinomas, mixed mullerian tumors, sarcomatoid form of sex cord stromal tumours, Krukenberg tumors associated with stromal reaction, and cellular fibroma. In sarcomas of the ovary, chondrosarcoma, malignant schwannoma, angiosarcoma, rhabdomyosarcoma are uncommonly noticed. Occasionally leiomyosarcoma is found as a teratomatous origin. However, leiomyosarcoma of nonteratomatous origin are infrequent. Leiomyosarcorna of non-teratomatous origin is also called as true leiomyosarcoma. We came across a case of this rate variety. Literature is reviewed to know the presentation and management of leiomyosarcoma of the ovary.
A 60-year-old female with history of post-menopausal period of 15 yr registered in this institute in August 1991 with chief complaints of lump in abdomen and generalised weakness of three months duration. Her past medical and obstetric history (3 full term normal deliveries) was uneventful. She was found anaemic on general examination. Abdomen was distended with main bulging over hypogastric and iliac areas. On examination, a mass was felt with ill-defined borders and had solid as well as cystic consistency. On per speculum examination, cervix could not be visualised and was felt to be pushed posteriorly. Hard, big mass with some cystic area occupying whole of the pelvic cavity was felt through rectal examination.
Her Haemoglobin was 6.8 gm%; rest all hematological and biochemical parameters were within normal limits. Except raised blood urea, other renal function and hepatic functions were normal. Chest X-ray showed multiple small rounded opacities suggesting metatases. Pyelogram suggested left hydronephrosis and hydroureter and right non-functioning kidney. Bladder was compressed to left side by extrinsic pressure. Ultrasonography of abdomen revealed bilateral hydronephrosis and echogenic mass in the pelvis. Normal uterus could be seen separately from the pelvic mass, which was pushed anteriorly. Ovaries could not be differentiated from the pelvic mass suggesting the ovarian origin of the mass. Paraaortic region and liver were free from metastasis. On cystoscopy, bladder was normal and there was impression of extrinsic pressure. Through examination under anaesthesia a solid-cystic mass was felt which was occupying almost whole of the pelvic cavity. Fine needle aspiration biopsy was taken from the pelvic mass through posterior fornix. Biopsy showed spindle cells in a hyalinized stroma along with pleomorphic cells and atypical mitoses suggesting the leiomyosarcoma most likely of ovarian origin, considering the clinical findings. Due to high blood urea level, sonography guided per cutaneous nephrostomy was done. As renal functions were deranged main treatment was surgical debulking to relieve pressure symptoms due to big ovarian mass. Since lung meatases were not symptomatic and renal functions were not normal, chemotherapy was not given as initial treatment. On exploration, tumour was found arising from the right ovary. Excision of mass with bilateral salpingo-oophorectomy and hysterectomy was done. Except slight adhesions to the pelvis and small bowel, mass could be resected completely. Post-operatively renal function came to normal within one week. Post-operative radiotherapy to pelvis was given upto 50 Gy to avoid local recurrence. At six months followup after radiotherapy, there was no evidence of recurrence on abdomino-pelvic sonography. On chest X-ray multiple lung metastases were observed with increase size. However, due to low haemoglobin and poor general condition systemic chemotherapy could not be given. Patient was asked to come for chemotherapy at next follow-up but did not turn-up. Patient died 18 months after treatment, noticed through letter correspondence.
Ovarian leiomyosarcornas present as large, soft, pale, fleshy mass. On gross examination these tumours are indistinguishable from other sarcomas. Usually these tumours are solid but cystic degeneration is often seen in large tumours. Microscopically tumour varies from well differentiated to highly pleomorphic sarcoma. Leiomyosarcoma differs from the benign counterpart in being hypercellular and by the presence of increased mitotic rate (greater than 10 per 10- high power fields). Mitotic activity varies from case to case. However, in most cases evidence of malignancy is degeneration. Cells tend to be spindle shaped and uniform, with abundant eosinophilic cytoplasm. Often cells are organised in whorled, interwoven bundle with area of pleomorphism. Nuclei are elongated. Occasionally a prominent area of ischemia is also observed.
Ovarian leiomyosarcoma is ambiguous in its genesis, since presence of identifiable smooth muscle has not been documented in the human ovary. However, smooth muscle cells are recognised in surrounding mesonephric elements and in the wall of blood vessels. Possibly, it is thought that uterine subserous leiomyomas may migrate from the site of origin and secondarily involve the ovary. Nevertheless malignant transformation of leiomyoma is rarely seen. There is difference in presentation suggesting the unlikelihood of leiomyoma to be the precursor of leiomyosarcoma. Leiomyomas are usually located in superficial tissue, while ieiomyosarcomas have predilection for deeper tissue.
Overall leiomyosarcomas account for 7% of all soft tissue sarcomas. These tumours are more common in females than in males. In the abdomen, common sites are omentum and mesentery. Two third of all leiomyosarcomas of retroperitioneal and intraabdominal origin occur in females with average age of 60 years at presentation, so was our case. Other rare sites of leiomyosarcoma are stomach, male breast, scrotum, bone, small intestine and vagina. In the literature these sites are mentioned as case reports.
Primary ovarian sarcomas comprise less than 3% of all ovarian neoplasm. The ratio of incidence of ovarian sarcoma to carcinoma is generally reported at 1:40. Primary ovarian sarcoma represents a heterogeneous group of ovarian tumours. Though encountered in all age groups, it primarily affects the post-menopausal patients. They are mostly associated with poor results and survival is generally anecdotal. These tumours usually present with non specific symptoms and signs and often produce symptoms of pressure effect on adjacent organs such as bladder and bowel.
Azoury et al analysed 2400 cases of ovarian tumour in the Emil Novak ovarian tumour registry and found 43 cases of primary ovarian sarcoma. They classified primary ovarian sarcoma into three categories according to the source of origin. They are teratoid, mesenchymal and mullerian (paramesonephric) type. Among 43 primary ovarian sarcomas two cases of leiomyosarcoma of teratoid origin and two cases of mesenchymal origin were found. Leiornyosarcomas of teratoid origin were unilateral and observed in younger age. Leiomyosarcoma of mesenchymal origin occurs in the elderly age group and more likely to metastasize. There were two cases of leiomyosarcoma of the mesenchymal origin. Both cases presented with ab'dominal metastases and despite radical surgery, they succumbed to disease.
Ovarian leiomyosarcoma of non-teratoid origin are extremely uncommon. About less than 20 cases could be traced from the available literature. However 14 other unpublished cases have been described. Tumour usually occurs in elderly women and mostly present with abdominal pain and mass. These tumours are mostly unilateral and often present in the advanced stage. Our patient also presented with weakness and abdominal lump of 3 months duration. At this stage, pressure changes in the kidney in form of hydronephrosis had taken place. Mean tumour mass in the 14 unpublished cases was 125 cm. In these cases, tumours were nodular as well as cystic in nature with areas of necrosis and haemorrhage. Leiornyosarcomas have also been associated with increased level of beta HCG.
Treatment recommended is radical surgery followed by adjuvant chemotherapy or radiotherapy. Prognosis is invariably poor and patients die with multiple wide spread distant metastases as well as big local disease and its pressure effect over other abdominal organs. Most patients expire within one year. Shakfeh and Woodruff reported two cases of ovarian leiomyosarcoma, one of these died in 12 months without receiving any specific treatment. Another case was treated with total hysterectomy with bilateral salpingo-oophorectomy followed by radiotherapy, who survived for 4 years. Only 2 of 16 reported cases have survived for 4 and 7 years; both were treated with radical surgery and post-operative radiotherapy. Kumar et al reported a case of 70 years female who presented with painful solid and cystic mass arising from the pelvis. Mass was explored and resected. Patient remained disease free for two years. It is opined that poor prognosis in this disease is due to its presentation in advanced stage. In majority of cases mass was too large to dissect radically and left untreated after biopsy or Exploration.
To increase survival in this disease multimodality treatment including radical excision or surgical debulking, post-operative radiotherapy to control local disease and routine chemotherapy after or concomitant to radiotherapy to avoid distant metastases in cases of large tumour volume is necessary. Pre-operative chemotherapy should also be tried to increase the operability of big pelvic disease.
We found that even in advanced cases surgical debulking gives good symptomatic relief and postoperative radiotherapy exerted local control, as no mass was detected at 6 months' follow up. However due to poor general condition of patient, systemic chemotherapy could not be started for distant metastases and early death could not be avoided.