Renal Hemangioblastoma with Mixed Mullerian tumor of endometrium: A tale of two rare primary tumors

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Pubblicato in:World Journal of Surgical Oncology (Jun 22, 2020), p. n/a
Autore principale: Setia, Aparna
Altri autori: Kumar, Devender, Bains, Lovenish, Sharma, Pallavi, Tempe, Anjali, Mallaya, Varuna
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Research Square
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024 7 |a 10.21203/rs.2.24455/v2  |2 doi 
035 |a 2539306783 
045 0 |b d20200622 
100 1 |a Setia, Aparna 
245 1 |a Renal Hemangioblastoma with Mixed Mullerian tumor of endometrium: A tale of two rare primary tumors 
260 |b Research Square  |c Jun 22, 2020 
513 |a Journal Article 
520 3 |a Introduction: Renal hemangioblastoma (RH) is a very rare benign tumor. Hemangioblastoma most commonly occur in the central nervous system (CNS) and only few cases of RH have been reported as they occur most commonly as asymptomatic masses found incidentally. Mixed Mullerian Tumor (MMT) of the uterus is a rarer and aggressive form of uterine malignancy. The detection of two primary rare tumors incidentally is a rare entity. Case presentation: A 50 years female presented with abnormal uterine bleeding which on endometrial sampling was diagnosed as a rare variety of endometrial cancer i.e., MMT or uterine carcinosarcoma. On preoperative imaging, a renal mass was also detected which was highly vascular and was mimicking Renal Cell Carcinoma (RCC). Fine needle aspiration cytology (FNAC) was done from the renal mass to differentiate between RCC and metastasis but it showed only blood cells. Patient underwent staging laparotomy for endometrial cancer and frozen section examination of the renal mass which was inconclusive with few atypical cells and thus patient underwent radical nephrectomy too. Histopathological examination revealed it to be a RH which is a very rare benign tumor. Discussion: RH is a rare benign tumor which doesn’t require any treatment in majority of the patients. Only 26 cases of RH outside the CNS have been reported till date. MMT is a rare aggressive uterine tumor with an incidence of 1-2 % of all uterine neoplasms, which metastasizes early and thus early identification and treatment is the key. RH needs to be differentiated from RCC to avoid over treatment. Morphological findings are similar in both, preoperative FNAC, PET scan and intraoperative frozen section can be utilized to differentiate in between two in well circumcised tumors and in high suspicion. Occurrence of renal mass as an incidental finding in the preoperative work up of uterine malignancy directed us to the differentials of metastasis or another histologically distinct primary tumor. The presence of two rare primary tumors i.e., RH and MMT in the same patient which are unrelated is a rare entity. 
653 |a Endometrial cancer 
653 |a Metastasis 
653 |a Tumors 
700 1 |a Kumar, Devender 
700 1 |a Bains, Lovenish 
700 1 |a Sharma, Pallavi 
700 1 |a Tempe, Anjali 
700 1 |a Mallaya, Varuna 
773 0 |t World Journal of Surgical Oncology  |g (Jun 22, 2020), p. n/a 
786 0 |d ProQuest  |t Science Database 
856 4 1 |3 Citation/Abstract  |u https://www.proquest.com/docview/2539306783/abstract/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch 
856 4 0 |3 Full Text - PDF  |u https://www.proquest.com/docview/2539306783/fulltextPDF/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch