|Year : 2020 | Volume
| Issue : 1 | Page : 41-46
Rare Entity of Sarcomatous Nodule Co-Existing With Benign Ovarian Tumour − A Case Report and Review of Literature
Poonam Sachdeva, Anamika Jain, Mannan Gupta
Department of Obstetrics and Gynaecology, Lok Nayak Hospital, MAMC, New Delhi, India
|Date of Submission||15-Sep-2019|
|Date of Decision||21-Oct-2019|
|Date of Acceptance||04-Mar-2020|
|Date of Web Publication||30-Apr-2020|
Dr. Anamika Jain
Department of Obstetrics and Gynaecology, Lok Nayak Hospital, MAMC, New Delhi
Source of Support: None, Conflict of Interest: None
Coexistence of mural nodule with ovarian tumour is an extremely rare entity. We highlight the case of a 60 year old postmenopausal lady with a sarcomatous nodule co-existing with ovarian mucinous cystadenoma. She had presented with an abdominal mass increasing over 2 years associated with pain. Image and lab analysis showed a multiloculated cystic mass 40*30*12 cm with features suggestive of mucinous cystadenoma with borderline elevation of CA 125 - - 118. 4 IU/ml.. She underwent total abdominal hysterectomy with right sided ovariotomy with left salpigooopherectomy and infracolic omentectomy with bilateral pelvic lymph node sampling. Frozen section report showed cystic ovary with mucinous fluid with a 2.5 cm reddish mural nodule in its wall. Microscopic section showed mucin secreting columnar epithelium lining. Subepithelially, the nodule showed highly pleomorphic cells with large nucleus having corse chromatin 4–10 mitoses/hpf. Final histopathology report was suggestive of mucinous cystadenoma with malignant sarcomatous nodule. Following surgery she recieved 3 cycles of carboplatin, paclitaxel regimen chemotherapy and sustained well. On thorough review of literature, only 42 such cases of coexistence of malignant mural nodules with benign tumors have been found. Considering the malignant potential of such nodules they must not be overlooked and treated accordingly.
Keywords: Autoimmune diseases, mural nodule, ovarian tumor, preeclampsia, rheumatoid arthritis, tuberculosis
|How to cite this article:|
Sachdeva P, Jain A, Gupta M. Rare Entity of Sarcomatous Nodule Co-Existing With Benign Ovarian Tumour − A Case Report and Review of Literature. MAMC J Med Sci 2020;6:41-6
|How to cite this URL:|
Sachdeva P, Jain A, Gupta M. Rare Entity of Sarcomatous Nodule Co-Existing With Benign Ovarian Tumour − A Case Report and Review of Literature. MAMC J Med Sci [serial online] 2020 [cited 2020 Aug 3];6:41-6. Available from: http://www.mamcjms.in/text.asp?2020/6/1/41/283510
| Introduction|| |
Co-existence of sarcomatous nodules with benign tumours is very rare, however they may be associated with benign, borderline or malignant nodules. Amongst the malignant nodules anaplastic carcinomas are the most common followed by sarcomatous nodules, mostly found in age group 17–77 years with median age at 37 years. Over the past 40 years only 43 cases have been reported. Incidence of a mural nodule in an ovarian neoplasm is between 2-5 per million. It is important to correctly classify a mural nodule so as to triage patients in need of aggressive adjuvant chemotherapy.
The author would like to present the case of Mrs. X, 60 year old, Para 3 Live 3 post-menopausal lady of Indian origin presented to Out Patient Department with chief complaints of pain abdomen, having history of gradually increasing abdominal mass over the past two years. There were no associated bowel or bladder complaints.
There was no history of diabetes, hypertension, tuberculosis or any ovarian malignancy in the past or in the family.
On examination her general condition was fair, vitals stable, afebrile, no pallor or pedal oedema was present.
On Per abdomen examination, a 34 to 36 week size mass, reaching from pelvis up to epigastric region was palpated, mass was cystic in consistency, immobile and non-tender
On per speculum examination, cervix and vagina were healthy with no discharge. On per-vaginum examination, same mass could be felt through all fornices, but uterus could not be felt separately. On per rectal examination, same mass could be felt, rectal mucosa was free and no nodules could be felt.
All routine investigations were within normal limit.
CA 125 was markedly raised to 118.4 IU/ml (Normal range: 0–35 IU/ml)
Ultrasound of whole abdomen revealed, large multicystic loculated lesion extending from epigastric region to pelvis having multiple solid areas with thin and thick septations with few loculi measuring approximately 20, 12, 19 cm in cranio caudal length. Uterus, bladder and adnexa could not be separately visualised [Figure 1] and [Figure 2].
Contrast-enhanced computed tomography (CECT) whole abdomen revealed large multicystic lobulated lesions extending from pelvis to epigastrium 40 × 30 × 12 cm in size showing multiple thick and thin septations with features suggestive of large mucinous cystadenoma.
There was no evidence of any other abdominal finding [Figure 3].
After Pre-anesthetic check up, staging laparotomy was performed.
Per operative findings: No free fluid was found and uterus was normal size and left fallopian tube and ovary were healthy.
A large right sided 60 × 50 cm mucinous cystadenoma of ovary having multiple septations was found. 100 ml Saline was instilled in the right and left paracolic gutters, and withdrawn using a heparinized syringe and was sent for cytology. Ovarian mass excision was performed and sent for frozen section.
Frozen section report was suggestive of malignant neoplasm. Mass excision was followed by total abdominal hysterectomy (TAH) with left salpigooopherectomy with infracolic omentectomy with bilateral pelvic lymph node sampling.
Frozen section report showed Cystic ovary measuring 20 × 18 × 12 cm. On cut section there were multiloculated areas showing mucinous secretions. Wall of ovary showed a reddish brown 2.5 cm nodule with friable appearance. Microscopic sections showed cystic areas lined by mucin secreting columnar epithelium. Subepithelially, the nodule showed highly pleomorphic tumour cells, large nucleus, coarse chromatin and mitoses of 4–10 per high power field. There were areas of necrosis. On ihc, mucinous lining of cyst wall was positive for CK and tumour cells in subepithelium were positive for vimentin.
Final histopathology report showed ovarian mucinous cystadenoma with 2.5 cm sarcomatous nodule.
Uterus, left fallopian tube and ovary were normal. Cervix showed chronic cervicitis with squamous metaplasia. Cystic glandular hyperplasia present. Myometrium unremarkable. Labelled common ileac lymph nodes showed reactive lymphoid hyperplasia. Omentum showed mild vascular congestion.
Bilateral paracervix, parametrium and resected cervical margins were uninvolved.
Cytology was negative for malignant cells.
Patient was advised chemotherapy post-hysterectomy, but she declined the treatment. She developed the complication of enterocutaneous fistula 6 months later due to secondary deposits, for which she got surgically treated at a tertiary care hospital in Mumbai and got healed. She is receiving chemotherapy currently, is on carboplatin, paclitaxel regimen. Has received three cycles of chemotherapy till date. Patient is vitally stable and sustaining well after chemotherapy.
Mural nodules of different origins have been described in ovarian tumours in a number of reports. Sarcoma like mural nodules (SLMNs) are the most common ones having a favourable prognosis. Malignant nodules are less common, they may be foci of anaplastic carcinoma, sarcomatous or carcinosarcomatous nodules.
To distinguish between benign and malignant nodules is of extreme importance as the course of management varies. Malignant sarcomatous nodules have poorer prognosis and require adjuvant chemotherapy in contrast to SLMNs. Although both are similar in morphology but vascular invasion, ill demarcation, marked nuclear atypia, numerous atypical mitotic figures are helpful to distinguish between sarcomatous and SLMNs.
Sarcomatous nodules are usually larger, composed of fibrosarcoma, undifferentiated sarcoma and rhabdomyosarcoma. The nodules of anaplastic carcinoma have microscopic features of nests and sheets of large round malignant epithelial cells.
SLMNs represent reactive and self-limited lesions within a neoplasia.
As far as the Role of Chemotherapy goes,
Adjuvant chemotherapy is required for management of ovarian tumours with coexistent sarcomatous nodules or else prognosis may be grave.
The patients undergo cycles of chemotherapy with drugs such as Liposomal doxorubicin, Taxanes such as paclitaxel and alkylating agents such as carboplatin, nedaplatin.
Follow up- Post-surgery and chemotherapy, women are followed up 3 monthly for first two years. At each visit clinical examination and CA 125 levels are done. Imaging using CT scan is done in case patient develops new symptoms or shows raised CA 125 levels.
Occurrence of Coexistence of mural nodule with ovarian neoplasm is exceedingly rare with incidence of 2 to 5 per million.,,, Table showing cases reported over the past three decades [Table 1].
|Table 1 Review of literature of coexistence of mural nodule in ovarian tumors: management and follow up|
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In conclusion this case is reported to highlight the importance of careful and thorough examination of a mural nodule within a mucinous cystic tumour and to ascertain its benign or malignant nature so that proper management can be done, which includes chemotherapy and follow-up in case of malignant nodules.
Benign nodules have a better prognosis as compared to malignant ones.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Mathew M, Gonsalves H, Al-Azawi S, Saparamadu PA. Asymptomatic ovarian mucinous cystadenoma with a solid mural leiomyoma: case report and brief review. Sultan Qaboos Univ Med J 2013;13:127-131.
Hameed A, Ying AJ, Keyhani-Rofagha S, Xie D, Copeland LJ. Ovarian mucinous cystadenoma associated with mural leiomyomatous nodule and massive ovarian edema. Gynecol Oncol 1997;67:226-9. 10.1006/gyno.1997.4867
Lifschitz-Mercer B, Dgani R, Jacob N, Fogel M, Czernobilsky B. Ovarian mucinous cystadenoma with leiomyomatous mural nodule. Int J Gynecol Pathol 1990;9:80-85. 10.1097/00004347-199001000-00008
Huang TY, Chen JT, Ho WL. Ovarian serous cystadenoma with mural nodules of genital rhabdomyoma. Hum Pathol 2005;36:433-5. 10.1016/j.humpath.2005.01.003
McFarland M, Dina R, Fisher C et al.
Osteosarcoma as malignant mural nodule in ovarian mucinous neoplasms of intestinal type: report of 2 cases. Int J Gynecol Pathol 2015;34:369-73.
Desouki MM, Fadare O, Kanbour A et al.
Immunophenotype and K-RAS mutation in mucinous ovarian adenocarcinoma with mural nodule of high-grade sarcoma: case report. Int J Gynecol Pathol 2014;33:186-90.
Rahilly MA, Candlish W, Al-Nafussi A. Fibrosarcoma arising in an ovarian mucinous tumor: a case report. Int J Gynecol Cancer 1994;4:211-4.
Tsujimura T, Kawano K, Taniguchi M, Yoshikawa K, Tsukaguchi I. Malignant fibrous histiocytoma coexistent with mucinous cystadenoma of the pancreas. Cancer 1992;70:2792-6
Bruijn JA, Smit VT, Que DG et al.
Immunohistology of a sarcomatous mural nodule in an ovarian mucinous cystadenocarcinoma. Int J Gynecol Pathol 1987;6:287-93.
Ongkasuwan C, Taylor JE, Tang CK et al.
Angiosarcomas of the uterus and ovary: clinicopathologic report. Cancer 1982;49:1469-75.
Prat J, Scully RE. Ovarian mucinous tumors with sarcoma-like mural nodules: a report of seven cases. Cancer 1979;44:1332-44.
[Figure 1], [Figure 2], [Figure 3]