Journal of Cytology
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CASE REPORT  
Year : 2012  |  Volume : 29  |  Issue : 4  |  Page : 255-257
Giant primary ovarian fibrosarcoma: Case report and review of pitfalls


1 Department of Pathology, Medical College, Kolkata, India
2 Department of Pathology, B.S. Medical College, Bankura, India

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Date of Web Publication28-Nov-2012
 

   Abstract 

Primary ovarian fibrosarcomas are very uncommon neoplasms; less than one hundred cases have been reported. Diagnosis can be difficult to establish and other similar appearing mesenchymal lesions must be ruled out. A 23 year old nulliparous woman presented with a huge right ovarian tumor (25 × 17 × 12 cm). Ultrasonography guided aspiration was performed and a strong possibility of fibrosarcoma was suggested based on cytomorphological findings. No epithelial element was noted. She underwent surgical excision which confirmed the diagnosis of primary ovarian fibrosarcoma (the largest till date to our knowledge). Immunohistochemical analyses showed vimentin and CD 34 positivity. We speculate that ovarian fibrosarcoma at the preoperative examination might be considered in the differential diagnosis whenever a unilateral, heterogeneous, largely round solid ovarian lesion is detected.

Keywords: Fibrosarcoma; ovarian neoplasm; sarcoma

How to cite this article:
Ray S, Biswas BK, Mukhopadhyay S. Giant primary ovarian fibrosarcoma: Case report and review of pitfalls. J Cytol 2012;29:255-7

How to cite this URL:
Ray S, Biswas BK, Mukhopadhyay S. Giant primary ovarian fibrosarcoma: Case report and review of pitfalls. J Cytol [serial online] 2012 [cited 2019 Dec 11];29:255-7. Available from: http://www.jcytol.org/text.asp?2012/29/4/255/103946



   Introduction Top


Ovarian neoplasms are common with surface epithelial tumors being the commonest overall and germ cell tumors in children. Fibrosarcoma is a rare entity in the ovary; less than one hundred cases have been reported so far. Isolated cases of unilateral primary fibrosarcoma of the ovary [1],[2] and a rare case of bilateral ovarian fibrosarcoma have been reported. [3] It has also been reported in association with the basal cell nevus syndrome [4] and Maffucci syndrome. [5] A case of primary ovarian fibrosarcoma is being presented because of its rarity, giant size and possible cytological and histological diagnostic dilemmas and pitfalls.


   Case Report Top


A 23 year old unmarried nulliparous female without any feminization or masculinization features presented with abdominal distension, intermittent pain abdomen and significant weight loss. Abdominal ultrasonography (USG) revealed a large, solid mass in the right adnexa. The contralateral ovary was normal. No lymph-node enlargement was detected. Other abdominal viscera were unremarkable. Significant ascites was present. Serum α-fetoprotein and chorionic gonadotropin levels were not raised.

USG guided aspiration yielded blood mixed material showing clusters of highly pleomorphic oval to spindle shaped cells with indistinct cell borders [Figure 1]a and atypical mitotic figures. A dissociated cell pattern was also observed at places [Figure 1]b along with few bizarre nuclei [Figure 1]c. No evidence of irregular branching or papillary configuration was seen. Psammoma bodies were absent. No acinar pattern or intracytoplasmic mucin was noted. There was no picket fence appearance as seen in mucinous neoplasms or coffee bean shaped nuclei of granulosa cell tumors. Call-Exner bodies were not found. Absence of abundant clear cytoplasm ruled out dysgerminoma. A cytological diagnosis of primary pleomorphic/ spindle-shaped sarcoma, possibly high grade fibrosarcoma, was proposed. Histological confirmation was suggested after taking adequate sections to rule out any malignant epithelial components missed due to sampling error that may occur in aspirated malignant mixed mesodermal tumors. Peritoneal fluid cytology was non-contributory.

Following surgical excision, grossly, the right ovary measured 25 × 17 × 12 cm with a smooth external surface [Figure 1]d. Sectioning revealed a solid, firm, grayish mass containing areas of hemorrhage and cystic degeneration. Histologically, the tumor was composed of spindle cells arranged in sheets and intersecting fascicles creating a diffuse herringbone appearance. There was mild to moderate nuclear atypia with enlarged, hyperchromatic, and oblong pleomorphic nuclei with prominent nucleoli. The tumor diffusely involved the entire ovarian stroma with no normal structures remaining. No epithelial component could be identified even after thorough sectioning. A formal mitotic count was carried out on all representative sections of the tumor by recording number of mitotic figures in 50 consecutive high-power fields (HPF) and calculating the average per 10 HPF. The mitotic count varied from 10 to 12 per 10 HPF. These features, particularly the raised mitotic activity, were diagnostic of fibrosarcoma. This was the largest ovarian fibrosarcoma reported till date to the best of our knowledge. Reticulin stain showed fibers separating individual neoplastic cells. Immunohistochemical stains were positive for vimentin and CD34.
Figure 1: (a) Clusters of pleomorphic spindle cells with indistinct cell borders (Pap, ×100). (b) Oval to spindle shaped cells with bizarre nuclei (arrow) (Pap, ×100)

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   Discussion Top


Primary fibrosarcomas of the ovary are rare and may arise de novo or as a result of malignant change in a pre-existing ovarian fibroma. [6] Guided aspirations are done to plan preoperative treatment protocols. USG guided aspiration in our case showed clusters of highly pleomorphic oval to spindle shaped cells with indistinct cell borders. Atypical mitotic figures along with few bizarre nuclei were also observed. No evidence of irregular branching or papillary configuration observed in serous neoplasms was seen. Psammoma bodies were also absent. No acinar pattern and intracytoplasmic mucin or vacuoles resulting in picket fence appearance encountered in mucinous neoplasms were seen. Coffee bean shaped nuclei and Call-Exner bodies of granulosa cell tumors were not found. Absence of abundant clear cytoplasm ruled out dysgerminoma. A cytological diagnosis of primary pleomorphic/spindle-shaped sarcoma, possibly high grade fibrosarcoma, was proposed considering the morphology, radiological solid appearance, age, absence of feminization or masculinization features with normal serum α-fetoprotein and chorionic gonadotropin levels.

Criteria for the diagnosis of fibrosarcoma of ovary were established by Prat and Scully. [7] They identified mitotic count as the most important feature for distinguishing between benign and malignant lesions. When they separated the tumors into two groups - cellular fibromatous lesions with mitotic counts up to 3 per 10 HPF (11 cases); and those with counts exceeding 3 per 10 HPF (6 cases) - a significant difference in survival was observed. The degree of cytologic atypia was a less reliable prognostic indicator. Although in their study, the lesions selected were pure fibromas showing no evidence of thecomatous differentiation, in a later review, Young and Scully [8] suggested that, in the absence of established criteria for malignancy, the same criteria could be applied to fibrothecomatous lesions. Despite the apparent reliability of mitotic counts in differentiating fibrosarcomas from cellular fibrothecomas, a few examples exist in which cellular fibrothecomatous lesions demonstrating low mitotic activity show an unexpected aggressive behavior. [9] Tsuji et al.[10] established that the MIB-1 (Ki-67) labeling index (LI) in atypical fibromatous lesions of the ovary was reflective of the potential aggressiveness of the tumor. MIB-1 LI for cellular fibromas ranged from 0.5 to 4.0 with a median of 2.3, while that for fibrosarcomas ranged from 3.0 to 10.8 with a median of 6.6. MIB-1 LI was related to the mitotic count, and their results may support a formal mitotic count as a diagnostic tool, especially in cases with a count of three to four mitoses per 10 HPF.

Primary ovarian fibrosarcomas, although rare, must be considered in the differential diagnosis of a unilateral, heterogeneous, solid ovarian lesion. Although usually encountered in the peri/postmenopausal age group, they can occur at any age as seen in our case. They are often underdiagnosed pre-operatively, resulting in diagnostic dilemmas. Cytological suspicion benefits the patient with proper hormonal assessment, intra-operative surgical excision protocols and postoperative management. However, a cautious approach is warranted categorically ruling out other common possibilities as discussed.

 
   References Top

1.Stellato G, Di Bonito M, Tramontana S. Primary fibrosarcoma of the ovary. Acta Obstet Gynecol Scand 1995;74:649-52.  Back to cited text no. 1
[PUBMED]    
2.Ikegami A, Ono I, Harada T, Ogura Y, Sumori K, Kouno K, et al. A case of fibrosarcoma of the ovary. Nihon Sanka Fujinka Gakkai Zasshi 1996;48:1177-9.  Back to cited text no. 2
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3.Ghosh AK. Bilateral fibrosarcoma of the ovary following hysterectomy. Am J Obstet Gynecol 1972;112:1136-8.  Back to cited text no. 3
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4.Kraemer BB, Silva EG, Sneige N. Fibrosarcoma of ovary: A new component in the nevoid basal-cell carcinoma syndrome. Am J Surg Pathol 1984;8:231-6.  Back to cited text no. 4
[PUBMED]    
5.Christman JE, Ballon SC. Ovarian fibrosarcoma associated with Maffucci's syndrome. Gynecol Oncol 1990;37:290-1.  Back to cited text no. 5
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6.Talerman A. Non-specific tumors of the ovary, including mesenchymal tumors and malignant lymphoma. In: Kurman RJ, editor. Blaustein's pathology of the female genital tract. 4th edition. Springer: New York; 1993. p. 916-7.  Back to cited text no. 6
    
7.Prat J, Scully RE. Cellular fibromas and fibrosarcomas of the ovary: a comparative clinicopathological analysis of seventeen cases. Cancer 1981;47:2663-70.  Back to cited text no. 7
[PUBMED]    
8.Young RH, Scully RE. Ovarian sex cord-stromal tumors. Problems in differential diagnosis. Pathol Annu 1988;23:237-96.  Back to cited text no. 8
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9.McCluggage WG, Sloan JM, Boyle DD, Toner PG. Malignant fibrothecomatous tumor of the ovary: diagnostic value of antiinhibin immunostaining. J Clin Pathol 1998;51:868-71.  Back to cited text no. 9
    
10.Tsuji T, Kawauchi S, Utsunomiya T, Nagata Y, Tsuneyoshi M. Fibrosarcoma versus cellular fibroma of the ovary: a comparative study of their proliferative activity and chromosome aberrations using MIB-1 immunostaining, DNA flow cytometry and fluorescence in situ hybridization. Am J Surg Pathol 1997;21:52-9.  Back to cited text no. 10
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Correspondence Address:
Sumit Ray
'Anjali', 6/1 J, Raja Bagan Lane, P.O. Ghugudanga, Kolkata 700 030, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9371.103946

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