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CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 134-136
Primary rhabdomyosarcoma in ovary – Pathologist clinches it all


1 Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Surgical Oncology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
4 Department of Medical Oncology, Dr. BRA Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication22-Mar-2018
 

   Abstract 


Sarcomas are extremely complex and heterogeneous group of malignancies. However, exact categorization of the type of sarcoma is essential for the individualized approach for a given patient. It is mandatory that sarcomas should be treated in tertiary care centers with good pathology support and expertise. Here, we present an apt example of a young girl with large abdominal mass which was diagnosed as ovarian rhabdomyosarcoma (RMS). Besides, her excellent response to RMS regimen further reinforces the findings.

Keywords: Ovarian tumors, rhabdomyosarcoma, sarcoma

How to cite this article:
Vanidassane I, Kumar S, Gunasekar S, Mathur SR, Phulware R, Rastogi S. Primary rhabdomyosarcoma in ovary – Pathologist clinches it all. Indian J Pathol Microbiol 2018;61:134-6

How to cite this URL:
Vanidassane I, Kumar S, Gunasekar S, Mathur SR, Phulware R, Rastogi S. Primary rhabdomyosarcoma in ovary – Pathologist clinches it all. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Aug 10];61:134-6. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/134/228177





   Introduction Top


Rhabdomyosarcoma (RMS) is indubitably the most common soft-tissue sarcoma of childhood. However, primary ovarian RMS is an exceedingly rare malignancy which might present as a diagnostic dilemma for both clinicians and pathologists. It is pertinent to delineate the exact diagnosis as treatment of RMS is different from other tumors that happen in the ovary. While there are still some cases reported from the developed world, the data from developing countries are very sparse which could be mainly due to lack of evolved pathology services or lack of reporting. Here, we describe one such case with combined clinical and pathological perspective. This case report also highlights the weak referral system and need for the coordination between different health-care levels.


   Case Report Top


A 21-year-old unmarried female without any previous comorbidity presented to a local physician with the complaints of abdominal pain, loss of appetite, and loss of weight for the past 4 months. Documented physical examination showed left lower abdomen mass. Ultrasound abdomen and contrast-enhanced computed tomography (CECT) scan revealed abdominopelvic solid cystic lesion of 22.5 cm × 22.5 cm arising from the left ovary extending up to renal hilum, CA125 was 108 U/ml. With the suspicion of ovarian epithelial tumor, ovarian staging, including washings and omentectomy along with left salpingo-oophorectomy was done 3 months before presentation to referral oncology center in the vicinity. Ascitic fluid cytology was reported as metastatic adenocarcinoma. Histopathology reported outside was suggestive of suggestive of low-grade myxoid fibrosarcoma with neoplastic cells positive for smooth muscle actin (SMA), CD34, vimentin, and focal desmin.

After 1 month of resection, the patient had a recurrence of symptoms and a postoperative magnetic resonance imaging (MRI) evaluation showed 6.4 cm × 3.4 cm × 6.5 cm lesion in the left adnexa with solid cystic components.

Following this, the patient was referred to our institute. She presented to our Institute after 3 months of surgery with rapidly increasing abdominal distension and features of bowel obstruction and Eastern Cooperative Oncology Group performance status 4. She was admitted and started on supportive management. A provisional clinical diagnosis of germ cell tumor, low-grade myxoid fibrosarcoma, ovarian sarcoma, and secondary ovarian tumor was made. Reevaluation at our center with CECT scan showed a large 30 cm × 13 cm × 25 cm ill-defined solid cystic abdominopelvic mass lesion with irregular thick septations noted within the peritoneal cavity causing mass effect, displacing uterus, bowels, bladder along with subcutaneous nodule 2 cm × 2.3 cm seen in lower anterior abdomen wall [Figure 1]. On comparison with previous postoperative MRI, there was 100% increase in size of mass. Her other tumor markers were found to be normal (alpha-fetoprotein – 0.88 ng/mL, human chorionic gonadotropin – 1.2 IU/mL, and lactate dehydrogenase – 413 U/L). Chest imaging was normal at baseline and presentation to our center. To our surprise, subsequent morphological and immunohistochemical findings proved it as embryonal RMS. Our differentials thus narrowed down to RMS arising from teratoma or carcinosarcoma or primary RMS of the ovary. After complete pathological, clinical, and radiological evaluation, we made a final diagnosis of primary ovarian RMS group 4 and stage 4. The patient was planned for VAC (vincristine, actinomycin D, and cyclophosphamide) high-risk protocol. In view of poor PS and intestinal obstruction, vincristine was withheld in the first dose. She responded to therapy and bowel obstruction resolved in a week. The patient is currently going through treatment.
Figure 1: (a) Baseline computed tomography abdomen showing solid cystic mass arising from the left ovary; (b) magnetic resonance imaging showing mass after 1-month postsurgery with displacement of bowel loops; (c) computed tomography scan at our center showing increase in size of mass causing mass effect

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Gross examination revealed a specimen consist of multiple soft grayish and reddish brown pieces of tissue measuring together 26 cm × 12 cm × 7 cm. Cut surface was gray along with areas of hemorrhage and necrosis.

Microscopic examination showed a tumor comprising of malignant spindle cells arranged in fascicles with myxoid and necrotic areas. The tumor cells showed mild-to-moderate amount of nuclear pleomorphism and inconspicuous nucleoli. In one of the sections, the tumor was seen in continuity with the ovarian parenchyma [Figure 2]. The tumor cells were immunopositive for desmin, myogenin, and vimentin while immunonegative for Pancytokeratin, epithelial membrane antigen (EMA), inhibin, calretinin, SMA, DOG-1, and MIC-2. Based on the histomorphological and immunohistochemical findings, diagnosis of embryonal RMS of ovary was rendered.
Figure 2: (a) Tumor comprising of spindle-shaped cells with moderate anisonucleosis (H and E, ×400); (b) Tumor seen in continuity with normal ovarian parenchyma (H and E, ×200); (c and d) Tumor cells immunopositive for desmin and myogenin (immunohistochemistry, ×200)

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   Discussion Top


Primary ovarian RMS is an exceptionally rare tumor. In the past 5 decades, not more than 50 cases have been reported. Before diagnosing primary ovarian RMS, it is essential to rule out metastasis from other sites, RMS arising from carcinosarcoma or teratoma. Ovarian RMS emphasizes the fact that RMS can arise in the structures where striated muscles are not the part of normal organ. Although the cell of origin of this tumor is still not known, it has been hypothesized to stem from either the uncommitted stromal fibroblasts or from fibroblasts of the endometriotic stroma.[1] We did not find any other site of disease by clinicoradiological workup. Neither did we find any teratomatous/heterologous elements or carcinomatous component.

Guérard et al. reviewed literature and reported outcome of 14 cases of ovarian RMS and described ovarian RMS as “very rare and lethal ovarian neoplasms” in the wake of almost 100% mortality.[2] Furthermore, they reported a very wide age range of 13 months to 86 years, and more than half of tumors were outside ovary at presentation. Subsequently, Nielsen et al. reported review of 13 cases, of which 11 were embryonal histology. Apparent poor outcome was evident as out of 11 patients who were followed up, seven patients died within 3 years of treatment, reinforcing the poor outcome of these tumors.[3] Cribbs described 2 cases (one each of embryonal and alveolar subtypes) of pediatric ovarian sarcoma, metastatic at presentation and were treated with combined modality (surgery and chemotherapy). Both the patients were disease free on follow-up till 8 months.[4]

In a report from Pakistan, Qureshi et al. reported a 21-year-old girl with pleomorphic ovarian RMS, undergone surgery and refused chemotherapy and was well till the last follow-up.[1] Ezem et al. reported metastatic alveolar RMS of the ovary from Africa in a 13-year-old girl and the diagnosis was made after laparotomy; however, the patient was not given chemotherapy or radiotherapy postoperative and died of metastatic disease.[5]

We could find only one case previously reported in India of a 10-year-old girl where it was resectable, and after that, the patient was given chemotherapy and was disease free after 16 months of follow-up.[6]

In a nutshell, this tumor occurs in a wide range of age groups, presents usually in advanced stages with delayed presentation, is diagnosed only after surgery of primary, has apparently poor outcomes. However, having said this, it is still not known exact course of this disease, need for abdominal radiotherapy and ruling out the possibility that some of them might arise from  Fallopian tube More Detailss.

We also want to underscore the delay in presentation in our center and the lacunae in referral system in country like ours. Recently, Gulia et al. have proposed hierarchical medical oncology services model in India.[7] However, such model is unlikely to be successful in the presence of outdated information technology, poor referral services, and lack of coordination between the centers. Besides, in disease like sarcoma where there is extreme heterogeneity and involves immunohistochemical and molecular tests should be treated in tertiary care centers only. Thus, differing from Gulia et al., we feel that this hierarchy should not only be based on complexity of chemotherapy but also the diagnosed disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Qureshi A, Hassan U, Rehman R. Primary ovarian rhabdomosarcoma. BMJ Case Rep 2011;2011: pii: bcr0120113677.  Back to cited text no. 1
    
2.
Guérard 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. 2
    
3.
Nielsen GP, Oliva E, Young RH, Rosenberg AE, Prat J, Scully RE, et al. Primary ovarian rhabdomyosarcoma: A report of 13 cases. Int J Gynecol Pathol 1998;17:113-9.  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
    
5.
Ezem BU, Onyiaorah IV, Ukah CO. Primary ovarian rhabdomyosarcoma: A case report. Afrimedic J 2011;2:35-8.  Back to cited text no. 5
    
6.
Sangwan N, Nanda S, Chauhan MB, Sen R, Gupta V, Kadian V. Primary ovarian rhabdomyosarcoma. J Gynecol Surg 2012;28(6):428-30.  Back to cited text no. 6
    
7.
Gulia S, Sengar M, Badwe R, Gupta S. National cancer control programme in India: Proposal for organization of chemotherapy and systemic therapy services. J Glob Oncol 2017;3:271-4.  Back to cited text no. 7
    

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Correspondence Address:
Sameer Rastogi
Department of Medical Oncology, Dr. BRA Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_548_16

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