Setia Aparna, Kumar Devender, Bains Lovenish, Sharma Pallavi, Tempe Anjali, Mallya Varuna
Department of Obstetrics and Gynaecology, Maulana Azad Medical College, New Delhi, India.
Department of Surgery, Maulana Azad Medical College, New Delhi, India.
World J Surg Oncol. 2020 Jul 6;18(1):154. doi: 10.1186/s12957-020-01929-1.
Renal hemangioblastoma (RH) is a very rare benign tumour. Hemangioblastoma most commonly occurs 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 tumour (MMT) of the uterus is a rarer and aggressive form of uterine malignancy. The detection of two primary rare tumours incidentally is a rare entity.
A 50-year-old 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 tumour.
RH is a rare benign tumour which does not 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 tumour 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 RCC and RH; preoperative FNAC, PET scan, and intraoperative frozen section can be utilized to differentiate the two, in well-circumcised tumours and high index of 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 tumour. The presence of two rare primary tumours, i.e. RH and MMT in the same patient which are unrelated, is a rare entity.
肾血管母细胞瘤（RH）是一种非常罕见的良性肿瘤。血管母细胞瘤最常发生于中枢神经系统（CNS），而RH病例报道较少，因为其最常见的表现是偶然发现的无症状肿块。子宫混合性苗勒管肿瘤（MMT）是一种较为罕见且侵袭性强的子宫恶性肿瘤。偶然发现两种原发性罕见肿瘤是一种罕见情况。
一名50岁女性因子宫异常出血就诊，子宫内膜取样诊断为一种罕见的子宫内膜癌，即MMT或子宫癌肉瘤。术前影像学检查还发现一个肾脏肿块，该肿块血供丰富，类似肾细胞癌（RCC）。对肾脏肿块进行细针穿刺细胞学检查（FNAC）以鉴别RCC和转移瘤，但仅见血细胞。患者因子宫内膜癌接受分期剖腹手术，并对肾脏肿块进行了冰冻切片检查，结果不明确，仅见少数非典型细胞，因此患者也接受了根治性肾切除术。组织病理学检查显示为RH，这是一种非常罕见的良性肿瘤。
RH是一种罕见的良性肿瘤，大多数患者无需任何治疗。迄今为止，中枢神经系统以外的RH病例仅报道了26例。MMT是一种罕见的侵袭性子宫肿瘤，占所有子宫肿瘤的1 - 2%，早期发生转移，因此早期识别和治疗是关键。需要将RH与RCC鉴别以避免过度治疗。RCC和RH的形态学表现相似；对于边界清晰且怀疑指数高的肿瘤，术前FNAC、PET扫描和术中冰冻切片可用于鉴别两者。在子宫恶性肿瘤的术前检查中偶然发现肾脏肿块，使我们考虑到转移瘤或另一种组织学上不同的原发性肿瘤的鉴别诊断。同一患者同时存在两种不相关的罕见原发性肿瘤，即RH和MMT，是一种罕见情况。
