伴有肉瘤样去分化的未分化巨细胞型胆囊癌:一例报告并文献复习

Undifferentiated giant cell type carcinoma of the gallbladder with sarcomatoid dedifferentiation: a case report and review of the literature.

作者信息

Manouras Andreas, Genetzakis Michael, Lagoudianakis Emmanuel E, Markogiannakis Haridimos, Papadima Artemisia, Agrogiannis George, Gakiopoulou Hariklia, Kekis Panagiotis, Filis Konstantinos, Patsouris Efstratios

出版信息

J Med Case Rep. 2009 Mar 18;3:6496. doi: 10.1186/1752-1947-3-6496.

Abstract

INTRODUCTION

Undifferentiated gallbladder carcinoma is a rare entity. Among unusual types of undifferentiated gallbladder carcinoma, giant cell type carcinoma is infrequent and, moreover, very few cases of such neoplasms with osteoclast-like giant cells have been documented. We report a case of undifferentiated gallbladder carcinoma presenting an unusual immunophenotype that was shown to be of giant cell type with sarcomatoid dedifferentiation infiltrated by osteoclast-like multinucleated cells.

CASE PRESENTATION

An 84-year-old Greek man presented with right upper quadrant pain, high fever, rigors, anorexia and weight loss during the past month. Clinical examination revealed tenderness in the right upper abdominal quadrant and a palpable gallbladder. Blood tests showed elevated white blood-cell count and transaminases. Abdominal ultrasound and computed tomography demonstrated a markedly distended gallbladder, measuring 16 cm x 8 cm, with oedema and pericholecystic fluid, consistent with gallbladder empyema. After an open cholecystectomy and an uneventful recovery, the patient was discharged on the 4(th) postoperative day. On cut surface, a 2cm solid mass was identified, obstructing the lumen in the neck of the gallbladder. Histopathology and immunohistochemistry offered the diagnosis of an undifferentiated, giant cell type carcinoma of the gallbladder with sarcomatoid dedifferentiation infiltrated with osteoclast-like giant cells.

CONCLUSIONS

Undifferentiated, giant cell type carcinoma of the gallbladder with sarcomatoid dedifferentiation infiltrated with osteoclast-like giant cells is a very infrequent neoplasm. Controversy exists over its nature, as related knowledge remains incomplete. Thorough histopathological and immunohistochemical evaluation is imperative for diagnosis. Due to their rarity, the biological behaviour and prognosis of these tumours remain unclear.

摘要

引言

未分化型胆囊癌是一种罕见的疾病。在未分化型胆囊癌的不常见类型中,巨细胞型癌较为罕见,而且,仅有极少数此类具有破骨细胞样巨细胞的肿瘤病例被记录在案。我们报告一例未分化型胆囊癌病例,该病例呈现出一种不寻常的免疫表型,经证实为具有肉瘤样去分化且有破骨细胞样多核细胞浸润的巨细胞型。

病例介绍

一名84岁的希腊男性,在过去一个月出现右上腹疼痛、高热、寒战、厌食和体重减轻。临床检查发现右上腹压痛,可触及胆囊。血液检查显示白细胞计数和转氨酶升高。腹部超声和计算机断层扫描显示胆囊明显扩张,大小为16 cm×8 cm,伴有水肿和胆囊周围积液,符合胆囊积脓。在进行开放性胆囊切除术后,患者恢复顺利,术后第4天出院。在切面可见一个2cm的实性肿块,阻塞了胆囊颈部的管腔。组织病理学和免疫组织化学诊断为未分化型、巨细胞型胆囊癌,伴有肉瘤样去分化,并有破骨细胞样巨细胞浸润。

结论

伴有肉瘤样去分化且有破骨细胞样巨细胞浸润的未分化型、巨细胞型胆囊癌是一种非常罕见的肿瘤。由于相关知识仍不完整,其性质存在争议。彻底的组织病理学和免疫组织化学评估对于诊断至关重要。由于它们罕见,这些肿瘤的生物学行为和预后仍不清楚。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1701/2726484/8fcad6c0accf/1752-1947-0003-0000006496-1.jpg

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