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- B65636 year old man a with duodenal mass（M36Y，十二指肠肿块）
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病例来自国外网站（http://www.pathologyoutlines.com/caseofweek/case200781.htm），谨致谢！
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这个网站的好东东不少，再次向大家推荐！
Clinical History
A 36 year old man was admitted to an outside institution with a one month history of nausea, vomiting, diarrhea and abdominal pain. He also had a 30 pound unintentional weight loss, anorexia and fatigue. He did not experience early satiety, dysphagia, increase in abdominal girth or hematuria, urinary frequency or rash. His past medical history included a cholecystectomy.
男，36岁，恶心、呕吐、腹泻、腹痛一月，伴无意识体重减轻(30磅)、厌食和疲劳，无早饱、吞咽困难、腹围增粗或血尿、尿频和尿急。有胆囊切除史。
The physical exam was entirely within normal limits, with no palpable abdominal mass or lymphadenopathy. Laboratory tests included WBC 17.6, hematocrit 43.8% and platelets 513K. The electrolyte panel was within normal limits. An abdominal CT scan demonstrated a large mass arising from the distal third of the duodenum, with intra and extra-duodenal components (Figure 1). There was mild dilation of the proximal duodenum, suggesting obstruction. Radiologically, a gastrointestinal stromal tumor (GIST) was strongly considered in the differential diagnosis, because of the size and location.
PE均在正常范围，未触及腹部肿块或肿大淋巴结。实验室检查：白细胞17.6，,红细胞比容43.8%，血小板513K。电解质正常范围。腹部CT：十二指肠远端三分之一处有一个巨大包块（图1）。十二直肠近端轻度扩张，提示梗阻。鉴于肿瘤的大小和位置，放射学上主要考虑的鉴别诊断是GIST。
The patient underwent an en-bloc resection of the tumor, partial pancreatectomy, and abdominal and regional retroperitoneal lymphadenectomy.
行肿块切除+部分胰切除+腹部和局部腹膜后淋巴结切除术。
The mass grew into the duodenal lumen and measured 10.5 x 10 x 8 cm (Figure 2). It did not appear to be originating from a lymph node. On H&E, the tumor was predominantly composed of spindle cells with a storiform pattern (Figure 3). Necrosis was present, along with numerous large, hyperchromatic cells and occasional multi-nucleated cells, with a scattering of small lymphocytes (Figure 4, Figure 5).
肿瘤突入十二指肠腔内，大小10.5 x 10 x 8 cm（图2），不像起源于淋巴结。HE：肿瘤主要由席纹状排列的梭形细胞组成（图3）。可见坏死，伴大量大细胞，染色质深染，偶见多核细胞，及散在的小淋巴细胞（图4，图5）。
Tumor cells stained dimly for S100, but were negative for c-kit/CD117, HMB45, CD34, desmin and actin. The tumor cells were immunoreactive for CD21 and CD35 (Figure 6).
IHC：S-100弱阳性， c-kit/CD117、HMB45、CD34、desmin、actin均阴性，但CD21和CD35阳性(图6)。
What is your diagnosis?
诊断？
标签：胆囊 滤泡树突状细胞肉瘤 FDCs
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本帖最后由 于 2007-09-12 20:36:00 编辑
华夏病理/粉蓝医疗
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努力让人人享有便捷准确可靠的病理诊断服务。
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×参考诊断
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本帖最后由 于 2007-09-12 21:42:00 编辑
滤泡树突样细胞肉瘤
滤泡树突样细胞肉瘤（FDCs）罕见，Monda等1986年描述其特征：通常是无痛性、惰性肿块，中位年龄50岁（范围17-76岁），无性别差异。累及腹部可有疼痛，但通常无全身症状。可在透明血管型Castleman病基础上发生，或两病同时发生。通常累及淋巴结，以颈、腋和纵隔淋巴结为主。结外部位包括口腔、脾、肝、小肠、胰、腹膜、软组织和皮肤。发生在脾和肝者与EB病毒有关。
典型者呈局限性生长，偶有远处转移至肝或肺。Fletcher等的文献回顾中，与复发、转移和致死的因素似乎包括：肿瘤体积大（≥6cm）、位于腹腔内和凝固性坏死。
除非想到此肿瘤并检测FDC标记（CD21和CD35），否则很可能误诊。主要的临床/病理鉴别诊断为GIST，尤其是位于腹腔内者。GIST和FDCs有相似的组织学特征，包括：束状排列的梭形细胞，常常出现上皮样细胞，偶尔S-100阳性。然而GIST总是c-kit（CD117）阳性且CD34通常阳性。
其它鉴别诊断包括：
fibroblastic reticulum cell sarcoma
纤维母细胞网状细胞肉瘤(vimentin+, SMA+, desmin+, CD21-, CD35-)
interdigitating dendritic cell tumor
（并）指状树突状细胞，或译作“交错树突状细胞肿瘤”(S100+, vimentin+, CD21-, CD35-)
黑色素瘤；
其他肉瘤。
华夏病理/粉蓝医疗
为基层医院病理科提供全面解决方案，
努力让人人享有便捷准确可靠的病理诊断服务。
