A Signet Ring Cell Tumor of The Ovary, Diagnosed After Treatment of Hodgkin lymphoma The First Case Of The Literature Dr. Sakir Volkan Erdogan Bakirkoy Dr. Sadi Konuk Training and Research Hospital

Signet ring tumor of the ovary is extremely rare kind of ovarian tumor. In the English literature less than twenty cases were reported additionally, the patient is the first case signet ring tumor of the ovary diagnosed after treatment of Hodgkin lymphoma.

A 33 year old woman who had been diagnosed with Hodgkin lymphoma appeared to have a solid mass of 58x49 mm in the right adnexal region which was later diagnosed as signet ring tumor of the ovary.

Ramzy et al. reported in 1976 the first signet-ring stromal tumor of the ovary case and is now considered to fall within the spectrum of the thecoma-fibroma group of sex cord–stromal tumors including thecoma, fibroma, and sclerosing stromal tumor.

This tumor is benign that occurs over a wide age range, in the English literature the youngest case is a four-year-old girl He Y 2010.

Because the tumor does not associate with hormonal symptoms patients commonly reveal incidentally or present with non specific abdominal pain.

During the pathological examination this type of tumor is characterized by extensive cytoplasmic vacuolation of tumor cells, resulting in a “signet- ring” appearance.

Signet-Ring Cell

Abdominal ultrasonography computed tomography or magnetic resonance imaging can show this lesions in the adnexa. The tumors treated with surgical but there is no standard treatment

Hodgkin lymphoma is a rare B-cell malignant neoplasm affecting approximately 9000 new patients yearly. Staging of this disease is essential for the choice of optimal therapy.

The initial treatment for patients with Hodgkin lymphoma is based on the histologic characteristics of the disease, the stage at presentation, and the presence or absence of prognostic factors associated with poor outcome.

Treatment of Hodgkin lymphoma depends on the stage commonly receive combined-modality therapies that include abbreviated courses of chemotherapy followed by involved-field radiation treatment.

The association of Hodgkin Lymphoma with signet ring cell tumor of the ovary is no studied before in the literature.

Case report

A 33 year-old parity one woman who had received chemotherapy , radiotherapy 9 years ago and transplanted autologous bone marrow stem cell 8 years ago due to Hodgkin Lymphoma was diagnosed with a solid mass of 58x49 mm in the right adnexa region which was noticed during routine follow-up.

Physical examination was unremarkable. A Doppler ultrasound was performed. A 45 mm mass was noticed on the lateral of the right ovary.

On the MR, a solid lesion which was hypo intense on T1 weighted images , heterogeneous hyper intense on T2 weighted images was observed.

MR Imaging

MR Imaging

MR Imaging

MR Imaging

The abdominal computed tomography showed no pathology in the adnexal regions six years ago.

According to the laboratory CA125 was 52 iu/ml, CA15-3 was 21 iu/ml and CA19-9 was 1 iu/ml.

A laparoscopic unilateral Salphingo- Oophorectomy was performed. During the surgery the mass which originates from the right ovary was observed . Left ovary and left tuba was noted as normal.

Macroscopically, the tumor presented as a well-demarcated lump of 8x6x5 cm with a wall which was measured between 0.6-0.8 cm . Sectioning disclosed that tumor was irregular and contained cystic materials.

Signet Ring Cells

Immunohistochemically, clear vacuoles of the signet-ring-like cells were negative for Cytokeratins. The signet-ring cells showed diffusely positive immunoreactivity for calretinin and inhibin. KI 67 proliferation index is under %1.

Pathology-Calretinin(+)

Pathology-Inhibin (+)

The diagnosis of signet ring stromal ovary tumor was made after histomorphologic and immunohistochemical examination.

A signet ring-like appearance can be found in association with nonneoplastic and neoplastic processes such as; several varieties of malignant lymphoma, malignant melanoma, meningioma, gastrointestinal stromal tumors etc.

discussion

This report describes a case of signet ring stromal tumor of the ovary associated with Hodgkin lymphoma.

The main differential diagnostic disease is Krukenberg tumor which is commonly bilateral and nearly always displays epithelial components other than signet- ring cells, being positive for different cytokeratins as well as EMA and containing mucin within the vacuoles

If PAS, mucin, EMA, and cytokeratin is negative in the signet ring stromal tumor cells there is strong evidence against Krukenberg tumor.

The differentiation between Krukenberg tumour and signet ring stromal tumour is critical as Krukenberg tumour is malignant with a extremely bad prognosis and signet-ring stromal tumour is benign.

Krukenberg tumours are bilateral nearly 63% of the time and are often multinodular. On the contrary, all of the previously reported signet-ring stromal tumour have been unilateral and uninodular.

These two findings have been the only significant distinguishing characteristics to differentiate these tumours but Garet K et al. reported a case multinodular and bilateral signet ring stromal tumor of the ovary in 2010.

The primary characteristic separating signet-ring stromal tumor from the other ovarian sex-cord stromal tumors is the presence of non-mucin/non-lipid signet- ring cells and it is demonstrated By Sue et al. in 2003 that the ultrastructure of the vacuole in the signet-ring like cells consists of cytoplasmic pseudo inclusions of extracellular edematous matrix.

Including: general edema of cytoplasmic matrix, dilatation of mitochondria, invagination of the cell membrane by extracellular matrix are three possible mechanisms of vacuole formation have described previously in signet-ring stromal tumor by Dickersin et al. in 1995.

Signet-ring cell stromal tumour of the ovary must be differentiated from other stromal tumours of the ovary especially techoma-fibroma.

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