Rapidly recurring ovarian fibrosarcoma after optimal ... - iKnow

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Case report

Rapidly recurring ovarian fibrosarcoma after optimal debulking surgery in a 46- year- old woman

Hariyono Winarto ,

^1,2

Tantri Hellyanti,

^2,3

Sigit Purbadi ,

^1,2

Sutrisno Sutrisno

^1,2

To cite: Winarto H, Hellyanti T, Purbadi S, et al. BMJ Case Rep 2020;13:e234757.

doi:10.1136/bcr-2020- 234757

1Department of Obstetrics and Gynaecology, Dr Cipto Mangunkusumo Hospital, Central Jakarta, Indonesia

2Faculty of Medicine, Universitas Indonesia, Central Jakarta, Indonesia

3Department of Anatomical Pathology, Dr Cipto Mangunkusumo Hospital, Central Jakarta, Indonesia Correspondence to Dr Hariyono Winarto;

hariyono. winarto@ ui. ac. id Accepted 14 June 2020

SUMMARY

Ovarian fibrosarcoma is an extremely rare tumour with no universally accepted guidelines for treatment. We present a 46- year- old nulliparous woman with ovarian fibrosarcoma who mainly presented with a painful abdominal enlargement. Optimal debulking surgery was performed, and a specimen of the tumour was examined. A frozen section examined during surgery revealed spindle cell morphology, raising the suspicion of ovarian fibrosarcoma, which was later confirmed by immunohistochemistry staining. Our patient refused to undergo chemotherapy and died 3 months after surgery due to tumour recurrence. Although no clear consensus exists for administering chemotherapy for fibrosarcoma, some published case reports have shown a lower chance of recurrence and better prognosis in patients who undergo chemotherapy compared with our patient.

BACKGROUND

Primary spindle cell sarcoma is one of the least frequently reported tumours. Based on the Surveil- lance, Epidemiology and End Results (SEER) database, only 3299 cases of primary spindle cell sarcoma were identified in 1973–2017, and most of those cases originated from the respira- tory system.¹ Ovarian sarcoma is extremely rare, accounting for less than 3% of ovarian neoplasms.² To the best of our knowledge, fewer than 100 cases of primary ovarian sarcoma have been reported to date,³ further confirming the rarity of these cases.

Patients with ovarian fibrosarcoma usually have low survivability due to distant metastasis via the bloodstream and tumour recurrence⁴; therefore, this disorder remains poorly studied. Here, we present a case of an aggressive primary ovarian fibrosarcoma.

CASE PRESENTATION

A 46- year- old nulliparous woman was referred to our hospital with a solid ovarian neoplasm. The patient complained about an abdominal mass, which had grown remarkably large and painful during the last 6 months. The patient also reported abnormal uterine bleeding and constipation. A systemic examination revealed noticeable abdominal enlargement resembling 36 weeks of pregnancy (figure 1), and a pelvic examination showed a cystic mass in the right adnexa to the xiphoid process. The mass had an irregular surface and restricted mobility.

INVESTIGATIONS

An ultrasound examination showed uterus enlargement due to a 32×27 mm inhomogeneous solid mass suspicious of a leiomyoma of the uterine cavity, and a solid, inhomogeneous mass sized 188×159 mm and suspicious of a malignant solid ovarian neoplasm on the right ovary. Massive ascites and pleural effusion were also found during the examination (figure 2).

A thoracic X- ray to evaluate the pleural effusion revealed infiltrates in the middle to lower lobe of the left lung, suspicious of pneumonia and a differential diagnosis of a pneumonia- type metastasis (figure 3).

A CT of the whole abdomen with contrast revealed a septated cystic mass with malignant characteristics in the right adnexa; the mass measured 15.2×17.4×22.2 cm and showed perito- neal seeding. No pelvic or paraaortic lymph nodes showed enlargement; however, adnexal mass infil- tration was suspected in the right uterine body and in the hepatic flexure colon. Uterine myomatosis with a lipoleiomyoma component was found in the posterior uterine body, with the largest size of 10×7 cm (figure 4).

Figure 1 Clinical presentation of the patient.

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A tumour marker examination showed an increase in Cancer Antigen (CA) 125 (467.7 U/mL), while carcinoembryonic antigen (CEA) and CA 19.9 were normal. The collected data led to a suspicion of an advanced- stage ovarian cancer. The patient was scheduled to undergo surgery for tumour debulking surgery and for histological determination of the type of ovarian cancer, which would guide further treatment.

DIFFERENTIAL DIAGNOSIS

Tumour debulking surgery was done as planned. Opening of the peritoneum revealed approximately 1000 cc of serous haemor- rhagic ascites. On further exploration, we found a cystic mass, measuring 35×25×20 cm and with irregular surface, on the right ovary. The cranial part of the mass was adhered to the omentum in the subhepatic region. The posterior part of the mass was adhered to the colon, and the anterior part of the mass was adhered to the posterior corpus of the uterus. We also found several solid masses on the uterine surface, measuring 3–6 cm each. The left ovary and fallopian tube were normal in size and shape. Tumour implants varying in size from 5 mm to 20 mm were present on the mesentery and were resected for further exploration. Other intraabdominal organs: liver, diaphragm, spleen, peritoneum, gaster, duodenum, jejunum, ileum, caecum, appendix, transverse colon and sigmoid colon were normal.

There was no enlargement of bilateral pelvic lymph node and

paraaortic lymph nodes. These findings suggested a primary sarcoma of the ovary.

The mass was sent to the pathology laboratory for frozen sectioning, and the result was a mesenchymal malignancy tumour (fibrosarcoma). Specimens from the right ovarian tumour, uterus, left ovary and fallopian tube, omentum and the tumour implants on the mesenterium were sent to the pathology laboratory for further examination (figure 5).

Pathological examination of paraffin block sections of the mass showed a diffuse cellular tumour mass inside lobules sepa- rated by connective tissue. The tumour cells were spindle shaped and pleomorphic, with rough chromatin. Mitotic figures were found at a rate of 13 per 10 high- power field (HPFs), with extensive necrotic areas and calcification. Immunohistochem- istry staining did not show any specific differentiation: desmin, smooth muscle actin (SMA), S100 protein, murine double- minute type 2 (MDM2), epithelial membrane antigen (EMA), CAM5.2, AE1/3, CD117, DOG-1, cytokeratins (CKs) 7 and 19 and CD34 were negative, with low positive muscle- specific antigen (MSA), diffusely positive vimentin and positive Ki67 in

>20% of the tumour (figure 6). Tumour spreading was found in the cervix and uterine body, omentum and mesenteries. The result confirmed that the tumour was a fibrosarcoma.

Spindle cell histological characteristics are found in several tumours, including leiomyosarcoma, fibroma, fibrosarcoma, malignant peripheral nerve sheath tumour (MPNST) and monophasic synovia sarcoma; this emphasises the importance of distinguishing between these tumours. There is no specific marker for fibrosarcoma; therefore, fibrosarcoma is a diagnosis of exclusion after other spindle cell sarcomas have been eliminated as options. Characteristics of fibrosarcoma include:

(1) hyperchromatic spindled cells showing pleomorphism, (2) herringbone or storiform growth pattern, (3) no morphological features of myxofibrosarcoma, low- grade fibromyxoid sarcoma Figure 2 Ultrasonography results for the patient. (A) A solid mass

inside the uterine body, suspicious of a uterine leiomyoma. (B) A solid mass arising from the right ovary. (C) Ascites and pleural effusion.

Figure 3 The thoracic X- ray evaluation of the patient. Infiltrates were present in the middle to lower lobe of the left lung.

Figure 4 Extension of the tumour, as shown by CT of the whole abdomen with contrast.

Figure 5 Gross macroscopic findings of the (A) right ovary and (B) uterus.

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or sclerosing epitheloid fibrosarcoma, (4) no expression of histological markers other than minimal vimentin or SMA and (5) mitosis index >4 per 10 HPFs, with atypical cytology.^5–7

Leiomyosarcoma is a malignant tumour that arise from smooth muscle; therefore, smooth muscle features should be noticed in the immunohistochemical examination. By convention, to diagnose a leiomyosarcoma, two myogenic markers, which include desmin, SMA and MSA, must be highly expressed in the immunohistochemical examination.^7–9 Our patient’s immunohistochemical examination showed negative for desmin and SMA and low positive for MSA, and thus ruled out leiomyosarcoma.

S100 protein is a specific marker for MPNST. CD31, CD34 and von Willebrand factor immunostains suggested vascular malignancies, such as spindle cell angiosarcoma. EMA and CKs are two useful markers to differentiate sarcomatoid carcinomas.

E- cadherin and β-catenin positivity are associated with mesenchymal to epithelial transition, which are useful to diagnose synovial sarcoma, epitheloid sarcoma and leiomyoma. Synovial and epitheloid sarcomas mostly express CKs 7, 8, 18 and 19.

Positive CD34 is helpful to determine a secondary fibrosarcoma from either solitary fibrous tumour or dermatofibrosarcoma.^{8 10} Our patient’s immunohistochemical result showed negative for these markers, thus ruling out the possibilities of the mentioned tumours. As other tumours have been excluded, we can conclude that the tumour was ovarian fibrosarcoma.

OUTCOME AND FOLLOW-UP

We performed optimal debulking of the tumour. We did a total hysterectomy, left salpingo- oophorectomy, omentectomy and resection of the tumour implants on the mesenterium. The

patient was admitted to the intensive care unit after optimal debulking of the tumour. On observation, the patient’s condition deteriorated due to hypovolemic shock, suspicious of internal bleeding. A relaparotomy was performed, and 6000 cc of blood was found in the peritoneum. Haemostasis was performed to stop bleeding. The patient was carefully observed and showed a gradual improvement in her clinical condition. After a few days of observation, no complications arose and the patient was discharged.

A month after surgery, the patient went to the outpatient clinic for a follow- up evaluation and treatment planning. The patient’s condition was good, with no remarkable findings. Adjuvant chemotherapy with carboplatin and paclitaxel was planned, but the patient refused to undergo chemotherapy.

The patient returned to the outpatient clinic 3 months after surgery and complained of bloating, sudden abdominal enlargement, shortness of breath and constipation. An ultrasound examination revealed a new malignant mass and ascites in the pelvic area. The patient was admitted to the inpatient ward to alleviate her symptoms and was planned for palliative care.

Chemotherapy could not be administered due to the patient’s condition. After 2 days of hospital care, the patient died.

DISCUSSION

Spindle cell sarcoma consists of a group of malignant soft tissue tumours with potentially destructive growth and local recurrence and significant distant metastasis risk. It is characterised by spindle cell neoplasms. Spindle cell sarcoma is classified into different subtypes based on its morphology, immunophenotype, genetics and differentiation.⁸

Based on differentiation, spindle cell sarcoma consists of two subgroups: well differentiated and undifferentiated. Leio- myosarcoma, fibrosarcoma and myofibroblastic sarcoma are examples of well- differentiated spindle cell sarcoma, whereas synovial sarcoma is the most common undifferentiated spindle cell sarcoma.⁸

Ovarian fibrosarcoma is extraordinarily rare. Its origin is believed to be stromal cells around the sex cord of the ovarian follicles. Malignant transformation of a fibroma is another possible origin of ovarian fibrosarcoma. Patients with fibrosarcoma are usually menopausal and postmenopausal women who complain of pelvic pain, abdominal enlargement or a rapid- growing abdominal mass, but these women can also present with abdominal pain, vaginal bleeding or pelvic swelling.⁴ Summary of the reported ovarian fibrosarcoma cases can be seen in table 1.

Based on a study of 31 cases of ovarian fibrosarcoma, the median patient age diagnosed with ovarian fibrosarcoma was 49 years old.⁴ In later cases, ovarian fibrosarcoma can also present in children. The youngest reported case was 9 years old and associated with DICER1 syndrome.¹¹ Fibrosarcoma of the ovaries usually occurs unilaterally, typically large and predomi- nantly solid, with haemorrhage and extensive necrosis, capsular destruction and infiltrative margins with adhesion to other pelvic organs. Microscopically, fibrosarcoma of the ovaries has common characteristics of fibrosarcoma in other organs. Fibro- sarcoma is often associated with trisomy 12 or 18. Fibrosarcoma of the ovaries usually has a poor prognosis due to early distant metastasis and resistance to several adjuvant chemotherapies.^{4 5 12}

Unlike the case for epithelial ovarian cancer, no universally accepted staging procedures are established for gynaecological sarcoma. Prat and Scully¹⁰ concluded that the mitosis index is the most important factor for diagnosing a fibrosarcoma, as nucleus grading and cellular pleomorphism are unreliable. They Figure 6 Histological preparation of the mass showed spindle cell

morphology (A) on H&E staining (400× magnification). (B) Extensive necrosis area of the tumour on H&E staining (100× magnification). (C) Calcification in the tumour on H&E staining (40× magnification). (D) Positive Ki67 in >20% of the tumour on immunohistochemistry staining.

(E) Positive vimentin on immunohistochemistry staining. (F) Leiomyoma of the uterus from a uterine specimen.

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stated that benign cellular fibroma has a mitotic index of 1–3/10 HPFs, while a mitotic index of ≥4/10 HPFs should be defined as a malignant fibrosarcoma.¹⁰ This agrees with the findings for our patient, who had a mitotic index of 13/10 HPFs, defined as malignant, and who succumbed to the disease in only 3 months after optimal debulking surgery. However, a study by Huang et al⁴ showed that the mitotic index is not a sole prognosis predictor of fibrosarcoma of the ovaries. The study demonstrated that in patients with low mitotic index (<4), distant metastasis could also happen in a short period, and, therefore, suggested that there were other factors for patient’s survival.⁴

Guidelines for the treatment of ovarian fibrosarcoma have not yet been developed. Fibrosarcoma is treated with optimal surgery due to its non- chemotherapy sensitive and non- radiation sensitive nature, as also stated by Miles et al.^{7 13} However, some literature shows that chemotherapy or radiation can prolong the patient’s survival. The longest patient’s survival was reported by Choi et al¹⁴, which included two cases of primary ovarian fibrosarcoma with long survival following treatment with chemotherapy after surgery. One case was free from disease, without evidence of recurrence, at 10 years after diagnosis, and the other remained free from disease 5 years later. The chemotherapy

regimen used were adriamycin/cisplatin and etoposide/ifosfamide/cisplatin.¹⁴ Gynecologic Cancer InterGroup consensus for ovarian sex cord stromal tumours stated that chemotherapy after surgery is recommended in advanced stage or metastatic and relapse cancer, with bleomycin, etoposide and cisplatin regimen for three to six cycles or carboplatin/paclitaxel.^{15 16} However, this recommendation is made by studying granuloma cell tumours as the most common sex cord stromal tumours. It is unclear whether this recommendation can be applied to ovarian fibrosarcoma, since it belongs to a different subclassification.

Due to this tumour’s aggressive behaviour and high recurrence rate, adjuvant chemotherapy administration and selec- tion should be personalised for each patient. Huang et al² reported that the use of mesna, doxorubicin, ifosfamide and dacarbazine for ovarian fibrosarcoma could prolong patient survival.² Other literature has shown that combinations of paclitaxel and cisplatin; cisplatin, etoposide and ifosfamide;

adriamycin and cisplatin; or doxorubicin and ifosfamide were also effective as adjuvant chemotherapies for ovarian fibrosarcoma.^{14 17} Our patient refused to undergo chemotherapy, so we could not evaluate the effectiveness of adjuvant chemotherapy in our patient.

Table 1 Reported ovarian fibrosarcoma cases

Author Age Immunohistochemistry Surgery Other therapy Recurrent Survival

Huang et al² 46 Spindle cells morphology arranged in a storiform configuration. No conspicuous cellular atypia, haemorrhage or necrosis. Ki67 no data and mitotic figures >5 per 10 HPFs.

TAH, BSO and LND Chemotherapy (epirubicin/ifosfamide/

dacarbazine)

No 72 months (alive)

Ozdemir et al³ 50 Positive vimentin and negative for SMA and desmin. Ki67 30%–40%

and mitotic figures 5–6 per 10 HPFs.

TAH and BSO None No 6 months (alive)

García Jiménez et al⁵

55 Positive for vimentin and negative CD117 and SMA. Ki67 60% and mitotic figures <1–2 per 10 HPFs.

TAH, BSO and omentectomy

Chemotherapy (ifosfamide/adriamycin)

Yes (14 months, liver) 14 months (alive)

Gultekin et al⁶ 52 Positive for inhibin, calretinin and SMA, and negative for EMA, CK, desmin and CD10. Ki67 9% and mitotic figure 4 per 10 HPFs.

TAH and BSO None No 12 months (alive)

Grenier et al⁹ 52 Positive for vimentin and negative for h- Caldesmon, inhibin, desmin, myogenin, pan- keratin, p52 and S100. Ki67 60%–70% and mitotic figures >20 per 10 HPFs.

TAH and BSO None (alternative treatment:

high- dose vitamin C, glutathione, vitamin B₁₇ and stem cell transfusion)

Yes (3 months, omental and 7 months, pelvic and abdomen)

15 months (deceased)

Prat and Scully¹⁰

61 No data. Ki67 no data and mitotic figures 4 per 10 HPFs. TAH, BSO and omentectomy

Radiation Yes (18 months, liver) 18 months (deceased)

None Yes (1 month, sigmoid

colon)

4 months (deceased)

42 No data. Ki67 no data and mitotic figures 25 per 10 HPFs. TAH, BSO and

omentectomy None Yes (1 month, ureter) 13 months (alive)

65 No data. Ki67 no data and mitotic figures 10 per 10 HPFs. Oophorectomy None Yes (6 months, pelvis) 13 months (deceased)

73 No data. Ki67 no data and mitotic figures 7 per 10 HPFs. None Chemo (not specified) Yes (2 months, pelvis and peritoneum)

2 months (deceased)

None Yes (44 months, pelvis and

peritoneum)

48 months (deceased)

Choi et al¹⁴ 44 Positive for vimentin and negative for SMA and S100. Ki61 <1% and mitotic figures 17 per 10 HPFs.

Chemo

(Adriamycin/ cisplatin)

34 Positive for vimentin and negative for SMA and S100. Ki67 20% and mitotic figures 8 per 10 HPFs.

Chemo

(Etoposide/ ifosfamide/

cisplatin)

Ray et al¹⁸ 23 Positive for vimentin and CD34. Ki67 no data and mitotic figures

10–12 per 10 HPFs. Right oophorectomy None Not reported Not reported

Celýk et al¹⁹ 49 Positive for vimentin and S100 and negative for desmin. Ki67 not

reported and mitotic figures 4 per 10 HPFs. TAH, BSO and

omentectomy Chemo

(paclitaxel/ cisplatin) Yes (36 months, pelvis

and liver) 42 months (deceased)

Testa et al²⁰ 44 Positive for vimentin and inhibin. Ki67 no data and mitotic figures 7

per 10 HPFs. TAH, BSO and

omentectomy Chemo

(Ifosfamide/ adriamycin) No 50 months (alive)

50 Positive for vimentin and inhibin. Ki67 12% and mitotic figures 5–7

per 10 HPFs. TAH, BSO and

omentectomy None No 5 months (alive)

Fukuda et al²¹ 54 Positive for vimentin and weak positive for α-inhibin. Ki67 5.4%–8.2%

and mitotic figures 3–6 per 10 HPFs.

Right oophorectomy None Yes (14 months, pelvis) 22 months (alive)

Grauso et al²² 58 Positive for vimentin, SMA and calretinin and negative for CD34, CD3, CK and EMA. Ki67 20% and mitotic figures 7–8 per 10 HPFs.

None No 24 months (alive)

Melendez- Zajgla et al¹¹

9 Positive for vimentin and negative for inhibin. Ki67 no data and mitotic figure 4 per 10 HPFs.

Tumour excision (not reported)

Not reported Not reported Not reported

BSO, bilateral salpingo- oophorectomy; CK, cytokeratin; EMA, epithelial membrane antigen; HPF, high- power field; LND, lymphadenectomy; SMA, smooth muscle actin; TAH, total abdominal hysterectomy.

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The prognosis of ovarian fibrosarcoma is generally poor, and patients with this fibrosarcoma usually do not survive long after surgery. A study by Huang et al, which included 31 patients of ovarian fibrosarcoma showed the median overall survival of patients was 42 months and median disease- free survival of patients was 18 months.⁴ Huang et al² and Choi et al¹⁴ reported some cases of ovarian fibrosarcoma with long- term survival. However, no prognostic factors for ovarian fibrosarcoma have yet been established due to the rarity of the disease. Based on the largest ovarian fibrosarcoma study conducted by Huang et al, FIGO stage and treatment were the independent prognostic factors for survival. Earlier The Inter- national Federation of Gynecology and Obstetrics (FIGO)

stage at diagnosis (FIGO stage IA–IC) were associated with better survival compared with more advanced stage (HR 0.231; 95% CI 0072 to 0743). Total hysterectomy with bilateral adnexectomy and omentectomy with chemotherapy after surgery were preferred among other treatment combinations as it is associated with better survival.⁴

Our patient was diagnosed with advanced stage fibrosarcoma with high mitotic figures and Ki67, as reported before. These factors may contribute to the poor prognosis of our patient.

In addition, our patient did not undergo chemotherapy, rather pursued an alternative treatment with unclear regiments. Even though there is no strong evidence that supports the use of adjuvant chemotherapy or radiation therapy, available studies mentioned before support the administration of adjuvant chemotherapy for a better survival. Therefore, we recommend the administration of personalised adjuvant chemotherapy in ovarian fibrosarcoma cases due to the inadequate evidence that supports certain regiments.

Contributors HW and TH: concept. HW, TH, SP and SS: data collection or processing. HW and SS: literature search. HW: writing.

Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not- for- profit sectors.

Competing interests None declared.

Patient consent for publication Next of kin consent obtained.

Provenance and peer review Not commissioned; externally peer reviewed.

Patient’s perspective

I remember how my wife was diagnosed with cancer. Her belly was getting so big that my family and friends thought she was pregnant.

She did a pregnancy test several times, but the results were negative, so I thought she was just gaining some weight. However, at some time later, she experienced pain in her belly and abnormal bleeding from her vagina. She also had some difficulty in passing stool. We decided to see a physician to have my wife treated.

The physician we saw referred us to an obstetrics and gynaecology specialist at a local hospital, and the obstetrics and gynaecology specialist said that the underlying cause of my wife’s problem was a mass in her ovary, and it was suspected to be malignant. My wife and I were shocked and could not think clearly. We heard that cancer is a serious disease and many of my acquaintances who had cancer had died miserably. Fortunately, our doctor was very supportive towards us and took really good care of us. We were referred to a bigger hospital to receive better care, because the facilities and resources there were better.

At the hospital, my wife was examined thoroughly to determine the extent of her tumour. After several examinations, the oncologists decided to do surgery to remove her tumour. The medical team had clearly stated that, after the surgery, my wife might have to undergo chemotherapy. We made several discussions with our family, and we agreed to the surgery.

I felt the surgery was very long and felt so worried. After 5 long hours of waiting, the oncology team said that the surgery went well and my wife’s tumour specimen was sent to the pathology laboratory for further examination. We received great care under the medical team during my wife’s hospitalisation. Although my wife had to undergo another surgery for her bleeding, our doctor had explained to us prior to the surgery that this might happen. We surrendered all to God. After the second surgery, my wife was getting better. A few days later, she was discharged from the hospital.

After the surgery, my wife felt a lot better. She did not feel any of the discomfort or pain she used to suffer. She was able to carry out her role in our family and society as well. We went to the polyclinic a month after my wife’s surgery to evaluate her condition. The doctor explained that the histopathological examination showed that the tumour my wife had was very rare. The doctor asked our permission to have my wife’s case published for the sake of medical education, and we agreed. The medical team also suggested that my wife undergo some courses of chemotherapy. Our oncologist had told us that my wife might have to undergo chemotherapy, but we decided not to go for chemotherapy. We thought my wife’s condition was good enough, and several people who had undergone chemotherapy seemed so miserable. Besides, we wanted to try an alternative treatment from a dukun recommended by our family.

We had already reserved an appointment with the dukun in 3 months. The medical team seemed disappointed, but he respected our choice.

Several weeks after our consultation, my wife was gradually weakened. She started to feel sleepy all the time, and her tummy enlarged rapidly. We made an appointment with the doctor in charge and my wife was admitted to the hospital. The oncologist told us that my wife’s tumour had come back and her condition was poor. The medical team would only manage my wife by relieving her discomfort, not to eradicate the disease anymore. On the second day of hospitalisation, my wife passed away.

I would like to express my gratitude to the medical team for their effort and care for my wife while she was fighting a battle with cancer. I wish for medical advancement so that people with ovarian cancer, like my wife, can have better outcomes.

Learning points

► Ovarian fibrosarcoma is a rare entity. Although the diagnosis criteria were established in 1981, fewer than 100 cases have been reported to date.

► Diagnosis of ovarian fibrosarcoma is made by exclusion.

Clinicians should exclude other tumours with a spindle cell appearance by immunohistochemistry staining.

► No clear evidence supports the use of adjuvant chemotherapy or radiation for treatment of ovarian fibrosarcoma. However, available studies suggest that administration of adjuvant chemotherapy may prolong patient survival.

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ORCID iDs

Hariyono Winarto http:// orcid. org/ 0000- 0002- 2081- 9238 Sigit Purbadi http:// orcid. org/ 0000- 0002- 5981- 5082 Sutrisno Sutrisno http:// orcid. org/ 0000- 0002- 3122- 7449 REFERENCES

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