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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 1  |  Page : 63-65

Giant ovarian fibrosarcoma: A rare case report


1 Department of Gynecological Oncology, SCI, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Pathology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
3 Department of Radio-diagnosis, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
4 Specialist Sub-Divisional Hospital, Hilsa Nalanda, Bihar, India

Date of Submission20-Jan-2022
Date of Acceptance14-Feb-2022
Date of Web Publication12-Feb-2022

Correspondence Address:
Sangeeta Pankaj
Department of Gynecological Oncology, SCI, Indira Gandhi Institute of Medical Sciences, Patna - 800 014, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jigims.jigims_7_22

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  Abstract 


Sex cord–stromal tumors are very rare ovarian tumors. Primary ovarian fibrosarcomas are a very rare type of sex cord–stromal tumors. They arise from superficial or deep connective tissues of fascia, tendon, periosteum, and scar. They can grow either slowly or rapidly forming a giant abdominal mass similar to epithelial tumors of the ovary. Fibrosarcomas are difficult to diagnose preoperatively. Tumor marker and radiological techniques play a trivial role in preoperative diagnosis of this rare variety of sex cord–stromal tumor. Often final diagnosis is made on histopathological and immunohistochemistry reporting. Histopathological features such as high mitotic count, nuclear atypia, and herringbone pattern arrangement of spindle cells confirm a diagnosis of malignant fibrosarcoma. Ki-67 index is considered a prognostic factor for fibromatous lesions of the ovary showing aggressive nature of tumor. We report a rare case of giant ovarian fibrosarcoma in a 40-year-old woman whose diagnosis was made histopathologically due to rarity of tumor.

Keywords: Histopathology, IHC, Ki-67, ovarian fibrosarcoma, sex cord–stromal tumors


How to cite this article:
Kumari P, Kumari S, Rani J, Abhilashi K, Atreya K, Kumar D, Choudhary V, Pankaj S, Jaiswal S. Giant ovarian fibrosarcoma: A rare case report. J Indira Gandhi Inst Med Sci 2022;8:63-5

How to cite this URL:
Kumari P, Kumari S, Rani J, Abhilashi K, Atreya K, Kumar D, Choudhary V, Pankaj S, Jaiswal S. Giant ovarian fibrosarcoma: A rare case report. J Indira Gandhi Inst Med Sci [serial online] 2022 [cited 2022 Jul 3];8:63-5. Available from: http://www.jigims.co.in/text.asp?2022/8/1/63/338371




  Introduction Top


Sex cord–stromal tumors are very uncommon ovarian tumors that usually occur in the first two to three decades of life.[1] These groups of ovarian tumors comprise 9% of ovarian neoplasms. Sex cord–stromal tumors are groups of tumors arising from granulosa cells, theca cells, Sertoli cells, Leydig cells, and fibroblasts of stromal origin. Depending upon cells of origin, sex cord–stromal tumors have different histopathologic subtypes which have both benign and malignant properties.[2] Because of the rarity of these tumors, less information is available. This subset of ovarian tumors manifests variable clinicopathologic features and biologic behavior, which often pose a diagnostic challenge for treating doctors.[3] Although levels of some tumor markers and sex hormones could sometimes be found abnormal, there is not any specific tumor marker to correctly diagnose sex cord–stromal tumors.[4] Ovarian fibromas belong to a sex cord–stromal group of tumors which constitute 3% of ovarian neoplasms, and their malignant counterpart is found even in less number of patients. Ovarian fibrosarcoma is a very rare malignant variety of ovarian tumors, usually seen in peri- or postmenopausal women. Fibrosarcomas can arise either de novo from the ovarian sex cord stroma, or rarely as a malignant transformation of a benign ovarian fibroma. Their origin is from superficial or deep connective tissues of fascia, tendon, periosteum, and scar. They can grow both slowly or rapidly and often found well circumscribed on histopathology.[5] Clinically, they manifest varied symptoms such as abdominal mass, pain, and heaviness in the lower abdomen.[6] The diagnostic criteria for fibrosarcoma include mitotic count and nuclear atypia which distinguishes benign lesions from malignant tumors. Based on nuclear atypia and mitotic count, this group of tumors is further divided into benign fibromas, mitotically active fibromas (MAFs), and fibrosarcomas. Benign counterpart of fibromas constitutes the most common type of spindle cell ovarian neoplasm, and is characterized by mild nuclear atypia and 3 or fewer mitotic figures per 10 high-power fields (HPFs).[7] MAF shows mixed features of both benign and malignant fibrosarcomas resembling borderline variety. It has a high degree of mitotic activity but lacks significant cytological atypia. MAF is considered a benign tumor with a favorable outcome. On the other hand, malignant fibrosarcomas show marked nuclear atypia and a higher degree of 4 or more mitotic figures per 10 HPFs.[6] Radiographically, it presents as a large unilateral heterogeneous solid ovarian mass with areas of hemorrhage and/or cystic degeneration. We present here a case report of giant fibrosarcoma in a 40-year-old woman.


  Case Report Top


A 40-year-old, P5L5, woman visited the gynecological oncology department of IGIMS, PATNA, with a complaint of abdominal distention for 6 months. Her menstrual history was normal with average flow and regular. Her vitals were within normal limit. On abdominal examination, a lump of 32-week size with mobility was found. On per vaginal examination, the cervix was healthy looking. Uterine size could not be assessed due to such a huge mass. Her tumor markers showed raised CA-125 level with a value of 152.7 U/mL. Other tumor markers were within normal limit. Contrast-enhanced computed tomography abdomen showed a 22 cm × 19 cm × 18 cm mass in the left adnexa with few nonenhancing cystic areas and multiple thin septa [Figure 1]. Mild congestion and tortuosity of the left adnexal vessels were present. The right ovary was normal. This patient after full evaluation for operative procedure underwent primary debulking surgery. Intraoperatively, a left ovarian mass of 25 cm × 22 cm found with torsion one round at pedicle was found [Figure 2]. The right ovary, uterus, and bilateral fallopian tubes were normal. total abdominal hysterectomy with bilateral salpingoophrectomy + total omentectomy was done. Para-aortic and pelvic lymph nodes were not palpable. Histopathological report on cut section showed a solid gray-white tumor of 20 cm × 18 cm × 16 cm with an area of hemorrhage and necrosis and with no capsular breach. The left ovarian section showed intersecting and herringbone pattern of oval-to-spindle cells intersected by thin fibrous septa. Mitotic activity was 5–10/HPF. Histological features consisted of fibrosarcoma [Figure 3]. The right ovary was unremarkable and the omentum was free of tumor. IHC was done which showed reticulin – positive, c-kit – negative, and Ki-67 – 35%. The patient was discharged after total stitch removal on the 14th day. Postoperative period was uneventful, and the patient is on regular follow-up till date in good condition.
Figure 1: Contrast-enhanced computed tomography abdomen pelvis showing a 22 cm × 19 cm × 18 cm mass in the left adnexa with few nonenhancing cystic areas and multiple thin septa, uterus normal size

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Figure 2: Intraoperative picture showing huge ovarian tumor (25 cm × 22 cm)

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Figure 3: Herringbone pattern of spindle cells arranged in sheets and intersecting fascicles with mild to moderate atypia and mitotic activity >4/0 high-power fields

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  Discussion Top


Primary ovarian fibrosarcomas are an uncommon rare type of sex cord–stromal tumors. Fibrosarcomas are difficult to diagnose, so other mesenchymal stromal tumors must be ruled out for exact diagnosis of fibrosarcomas. They are rare fibroblastic tumors of the ovary that typically has 4 or more mitotic figures per 10 HPFs as well as significant nuclear atypia in most of the cases.[7] Fibrosarcoma has become, in large part, a diagnosis of exclusion.[8] Fibrosarcomas are the most common ovarian sarcoma, occurring at any age, but most often seen in older women.[7] In our case, the patient was a 40-year-old woman. Tumor markers play a trivial role in preoperative diagnosis of this rare variety of sex cord–stromal tumors. In our case, all ovarian tumor markers were within normal limits except CA-125 that was also not too high. Similar studies conducted by ovarian tumor markers in normal range. Often final diagnosis is made on histopathological and IHC reporting. Fibrosarcomas can attain very large sizes just like epithelial tumors of the ovary. The tumor size in our case was 25 cm × 22 cm, whereas in the case report by Mathur et al.,[8] it was 6 cm × 6 cm and 20 cm × 20 cm, respectively. This is the largest size of primary ovarian fibrosarcoma reported so far in literature. Fibrosarcomas are typically large tumors that have often spread beyond the ovary at diagnosis, but in our case, there was no capsular breach found and omentum was also free of tumor even with such gigantism of tumor. Nowadays, immunohistochemical staining for Ki-67 antigen is used to assess the proliferative activity of various tumors and is considered a prognostic factor for fibromatous lesions of the ovary.[8] This index is reflective of the potentially aggressive nature of tumor.

Often help of other immunohistochemical markers such as vimentin, CD117, SMA, desmin, EMA, S-100, CD99, CD34, inhibin-alpha, estrogen receptor, and progesterone receptor in addition to the characteristic herringbone morphologic pattern is taken to establish a final diagnosis of fibrosarcoma.[6] In our case also, diagnosis was only confirmed after histopathology and immunohistochemistry report. Hence, a high index of suspicion of fibrosarcoma should be kept in mind while operating a huge abdominal mass of unilateral side.


  Conclusion Top


Primary ovarian fibrosarcomas are a very rare type of malignant ovarian tumors, but it must be considered a differential diagnosis of unilateral solid ovarian mass. This is a rare case report of such huge primary ovarian fibrosarcoma reported so far. These tumors are difficult to diagnose preoperatively. Tumor markers and radiological techniques sometimes might not help in getting preoperative diagnosis. Immunohistochemical assessment and histopathology report only confirm diagnosis. A high index of suspicion is always required to diagnose these rare subsets of tumors. This case report will add information available for management of this rare but aggressive tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, et al. Ovarian sex cord-stromal tumors. J Oncol Pract 2016;12:940-6.  Back to cited text no. 1
    
2.
Meinhold-Heerlein I, Fotopoulou C, Harter P, Kurzeder C, Mustea A, Wimberger P, et al. The new WHO classification of ovarian, fallopian tube, and primary peritoneal cancer and its clinical implications. Arch Gynecol Obstet 2016;293:695-700.  Back to cited text no. 2
    
3.
Lim D, Oliva E. Ovarian sex cord-stromal tumours: An update in recent molecular advances. Pathology 2018;50:178-89.  Back to cited text no. 3
    
4.
Haltia UM, Hallamaa M, Tapper J, Hynninen J, Alfthan H, Kalra B, et al. Roles of human epididymis protein 4, carbohydrate antigen 125, inhibin B and anti-müllerian hormone in the differential diagnosis and follow-up of ovarian granulosa cell tumors. Gynecol Oncol 2017;144:83-9.  Back to cited text no. 4
    
5.
Goldblum JR, Lamps LW, McKenney JK, Myers JL, editors. Soft tissues. In: Rosai and Ackerman's Surgical Pathology. 11th ed., Ch. 41. Philadelphia: Elsevier; 2018. p. 1810-915.  Back to cited text no. 5
    
6.
Grauso F, Messalli EM, Salzillo ME, Di Martino L, Falcone F, Orabona P, et al. Ovarian fibrosarcoma: Case report and latest trends in diagnostic and therapeutic management. Eur J Gynaecol Oncol 2015;36:742-5.  Back to cited text no. 6
    
7.
Omotoso AJ, Odusolu P, Ikpeme A, Irabor G, Nnoli M. Ovarian tumour in a tertiary institution in South Southern Nigeria. Int J Multidiscip Res Inf 2016;2:398-401.  Back to cited text no. 7
    
8.
Mathur C, Agarwal M, Kothari DC, Tailor SB. Primary ovarian fibrosarcoma – A rare case report. IOSR J Dent Med Sci 2016;1:65-8.  Back to cited text no. 8
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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