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 Table of Contents 
CASE REPORT
Year : 2022  |  Volume : 13  |  Issue : 2  |  Page : 190-192  

Sarcomatous nodules associated with mucinous neoplasms of ovary: A report of two cases


1 Department of Pathology, Maulana Azad Medical College, New Delhi, India
2 Department of Obstetrics and Gynecology, LNJP Hospital, New Delhi, India

Date of Submission08-May-2019
Date of Decision14-Jul-2019
Date of Acceptance26-Nov-2020
Date of Web Publication16-Sep-2022

Correspondence Address:
Varuna Mallya
Room No. 269, Pathology Block, Maulana Azad Medical College, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmh.JMH_77_19

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   Abstract 


Ovarian mucinous neoplasms (benign/borderline/malignant), one of the common surface epithelial tumours of ovary, at times can be associated with mural nodules. These mural nodules can either be sarcomatous, sarcoma like or anaplastic carcinomatous. The nodules can be single or multiple and appear solid, white to brown in color. Sarcomatous nodules tend to occur in older patients and microscopically are poorly circumscribed , comprising of a population of pleomorphic spindled cells. Two cases of sarcomatous nodules associated with ovarian mucinous neoplasm are being presented here.

Keywords: Anaplastic, nodule, ovary, sarcomatous


How to cite this article:
Kaur N, Chaudhary D, Singh V, Mallya V, Khurana N, Sachdeva P, Mala Y M. Sarcomatous nodules associated with mucinous neoplasms of ovary: A report of two cases. J Mid-life Health 2022;13:190-2

How to cite this URL:
Kaur N, Chaudhary D, Singh V, Mallya V, Khurana N, Sachdeva P, Mala Y M. Sarcomatous nodules associated with mucinous neoplasms of ovary: A report of two cases. J Mid-life Health [serial online] 2022 [cited 2022 Oct 6];13:190-2. Available from: https://www.jmidlifehealth.org/text.asp?2022/13/2/190/356193




   Introduction Top


Mucinous tumors of ovary arise from the surface epithelium and are classified as benign mucinous cystadenoma, atypical proliferative mucinous tumor and invasive mucinous carcinoma.[1] These mucinous neoplasms may rarely have mural nodules in their walls which are categorized as sarcomatous, anaplastic carcinomatous or sarcoma like mural nodules.[2] Amongst the three, anaplastic carcinoma nodules are the commonest. Seen mostly in postmenopausal women, these nodules have distinct microscopic and macroscopic features. The presence and nature of these mural nodules – benign versus malignant is of prime importance as their treatment and prognosis is poles apart.[3] Sarcoma like nodules representing a reactive phenomenon behave in a benign manner whereas carcinomatous or sarcomatous nodules have much worse prognosis. Their histogenesis is still debatable.

Two cases of sarcomatous nodules associated with ovarian mucinous neoplasm are being presented here.


   Case Reports Top


Case 1

A 66 year old female presented with complaints of abdominal pain and distension. A contrast enhanced computed tomography of the abdomen was done which revealed a multicystic lobulated lesion extending from the pelvis to the epigastrium measuring 40 cm × 30 cm × 12 cm with multiple thick and thin septations suggestive oflarge mucinous cystadenoma [Figure 1]. Her CA 125 was 118.4 IU/mL. Clinically, a diagnosis of mucinous cystadenoma was considered. Surgery was performed on gross examination the cyst was multiloculated with focal necrotic areas. At few places the wall showed multiple white to brown nodules [Figure 2]a. On histology, the cyst wall was lined by benign mucinous epithelium and subepithelialy showed highly pleomorphic spindled cells infiltrating the surrounding ovarian stroma [Figure 3]a. On IHC the overlying epithelium was positive for cytokeratin (CK) [Figure 4]a and the underlying spindle cells were positive for Vimentin [Figure 5]a. A diagnosis of mucinous cyst adenoma with sarcomatous nodules was established. The patient has currently received two cycles of carboplatin and paclitaxel regimen and is doing well.
Figure 1: Contrast enhanced computed tomography shows septate cystic lesion

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Figure 2: (a) Multiloculated cystic structure with multiple solid white nodules in the cyst wall. (b) Solid cystic mass with single nodule in the wall with frank areas of necrosis

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Figure 3: (a) Photomicrograph showing benign mucinous cuboidal to columnar unilayered epithelium, subepithelialy pleomorphic spindled cells infiltrating into the surrounding ovarian stroma identified (case 1) (H and E, ×100). (b) Photomicrograph showing stratified mucinous epithelum exhibiting hyperchromasia, increased nucleocytoplasmic ratio, with pleomrphic spindled cells seen in the subepithelium (case 2) (H and E, ×400)

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Figure 4: (a) Lining epithelium immunoreactive for cytokeratin (IHC, ×100). (b) Lining epithelium immunoreactive for cytokeratin (stroma is not reactive for cytokeratin) (IHC, ×400)

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Figure 5: (a) Spindle shaped cells present in the subepithelium are immunoreactive for Vimentin (whereas the lining epithelial is nonreactive) (IHC, ×400) (b) Spindle shaped cells present in the subepithelium are immunoreactive for Vimentin (whereas the lining epithelial is nonreactive) (IHC, ×400)

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Case 2

A 65 year old female presented with complaints of pain abdomen, abdominal distension, and nonpassage of stools for 4 days to the emergency room. She was taken up for an emergency laparotomy, and a large ovarian mass occupying the pelvic cavity pressing the bowel was seen. The mass was resected and received in the histopathology department. On gross examination, the mass was partly solid and cystic with gray white area [Figure 2]b. Histology of the lesion showed features of mucinous cystadenocarcinoma with pleomorphic sarcomatous nodule [Figure 3]b. The lining epithelium was immunoreactive for CK [Figure 4]b and the sarcomatous component for vimentin [Figure 5]b and negative for the epithelial markers. She was advised chemotherapy but refused and was lost to follow up.


   Discussion Top


In India, proportion of ovarian cancer varies from 6% to 7.7% of all female cancers.[4] Epithelial tumors account for 75% of all ovarian tumors.[5] Mucinous ovarian neoplasms represent 36% of all ovarian tumors, out of which75% are benign, 10% atypical, and 15% are malignant, respectively.[6] These ovarian mucinous neoplasms are rarely associated with mural nodules.[7] The nodules can be anaplastic carcinomatous, sarcomatous, or sarcoma like. Microscopic features along with immunohistochemistry helps in proper characterization. The mural nodules are usually seen in females in postmenopausal age group, frequently the nodule is single but rarely can be multiple. Grossly, the nodule is white to brown in color with necrotic areas. Here, both women were postmenopausal and presented with abdominal pain and distention. The first case had multiple sarcomatous nodules whereas the second case had a single nodule in the wall of the ovary. On microscopy, both the cases showed highly pleomorphic spindled cells infiltrating the surrounding ovarian parenchyma. These cells were immunoreactive for vimentin and negative for CK. Infiltrating into the surrounding stroma ruled out the possibility of sarcoma like nodule. A positivity of vimentin and negativity of CK in the spindled cells excluded carcinomatous nature of the mural nodules and eventually diagnosis of sarcomatous nodule was established. The mucinous ovarian neoplasm associated with mural nodules can be benign, borderline, or malignant. In our cases, the first case was associated with a benign mucinous neoplasm and the second case showed pleomorphism, atypia, stratification and invasion of the mucinous lining hence establishing a diagnosis of malignant mucinous neoplasm. Sarcoma like nodules probably represent a reactive process, whereas the sarcomatous or carcinomatous nodules arise as a result of divergent differentiation of a mucinous neoplasm or probably dedifferentiation.[6],[7]

The prognosis of patients with malignant mural nodules that is sarcomatous or carcinomatous type is usually poor. The mortality rate is up to 43% in 1.5 years,[7] whereas the 5 year survival rate for patient with FIGO stage 1 invasive mucinous ovarian carcinoma is 91%[8] Thus, careful identification and classification of mural nodule is required as carcinomatous and sarcomatous nodules require adjuvant chemotherapy. The current cases highlight the importance of thorough sampling of tumor to identify the correct entity and hence provide the correct plan of action.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Brown J, Frumovitz M. Mucinous tumors of the ovary: Current thoughts on diagnosis and management. Curr Oncol Rep 2014;16:389.  Back to cited text no. 1
    
2.
Seidman JD, Cho KR, Ronnette BM, Kurman RJ. Surface epithelial tumors of the ovary. In: Kurman RJ, Ellenson LH, Ronnette BM, editors. Blaustein's Pathology of the Female Genital Tract. 6th ed. New York: Springer.  Back to cited text no. 2
    
3.
Zhang Y, Zheng Y, Kai S, Li P. Ultrasonic and pathologic characteristics of ovarian mucinous cystic tumours with malignant mural nodules-two cases report. Medicine 2017;96:45.  Back to cited text no. 3
    
4.
Murthy NS, Shalini S, Suman G, Pruthvish S, Mathew A. Changing trends in incidence of ovarian cancer-the Indian scenario. Asian Pac J Cancer Prev 2009;10:1025-30.  Back to cited text no. 4
    
5.
Borderline mucinous cystic ovarian tumor with mural nodules (carcinosarcoma). Chang JS, Chua CC, Lee CC.Ann Saudi Med 2012:32:415-20.  Back to cited text no. 5
    
6.
Smith JA, Wolf JK. In: Dipiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM. Ovarian Cancer Pharmacotherapy: A Pathophysiologic Approach. 8th ed. New York: Mc Graw Hill; 2008. p. 2361 75.  Back to cited text no. 6
    
7.
Yang S, Wang L, Sun K. Ovarian mucinous cystic tumor associated with sarcomatous mural nodule and benign Brenner tumor: A case report and literature review. Medicine (Baltimore) 2019;98:e14066.  Back to cited text no. 7
    
8.
Riopel MA, Ronnette BM, Kurman RJ. Evaluation of diagnostic criteria and behaviour of ovarian intestinal type mucinous tumors-atypical prolifeartive (borderline) tumors and metastatic carcinomas. Am J Surg Pathol1999;23:617-35.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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