Alam Feroz, Alam Kiran, Qadri Shagufta, Ali Wasif Mohd
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh, Uttar Pradesh, India.
Department of Surgery, Jawaharlal Nehru Medical College, Aligarh, Uttar Pradesh, India.
BMJ Case Rep. 2014 May 21;2014:bcr2014204232. doi: 10.1136/bcr-2014-204232.
Among the group of small round cell tumours, there is a distinct and rare tumour known as desmoplastic small round cell tumour (DSRCT). DSRCT presents as multiple, widespread masses in the abdomen and pelvis and may be accompanied by extensive tumour implants throughout the peritoneum as the tumour is known to spread diffusely along serosal surfaces. We discuss a case of DSRCT in a 16-year-old boy who presented with abdominal pain since 2 years, a non-tender mass was palpable on the right upper quadrant of the abdomen, ultrasonographic and CT findings suggested hydatid cyst of liver. Laparotomy revealed multiple small peritoneal deposits along with a single mass in the liver. On histopathology, the lesion was found to be neoplastic and composed of predominantly clusters of small round blue cells, in a desmoplastic stroma; tumour cells were diffusely positive for cytokeratin, vimentin and neuron-specific enolase, thus confirming the diagnosis of DSRCT.
在小圆形细胞肿瘤组中，有一种独特且罕见的肿瘤，称为促纤维增生性小圆形细胞肿瘤（DSRCT）。DSRCT表现为腹部和盆腔内多发、广泛的肿块，并且可能伴有整个腹膜广泛的肿瘤种植，因为已知该肿瘤沿浆膜表面弥漫性扩散。我们讨论一例16岁男孩的DSRCT病例，该男孩自2年前开始出现腹痛，在右上腹可触及一个无压痛的肿块，超声和CT检查结果提示肝包虫囊肿。剖腹探查发现多个小的腹膜沉积物以及肝脏中的单个肿块。组织病理学检查发现该病变为肿瘤性病变，主要由小圆形蓝色细胞簇组成，位于促纤维增生性基质中；肿瘤细胞细胞角蛋白、波形蛋白和神经元特异性烯醇化酶弥漫性阳性，从而确诊为DSRCT。
