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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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Diagnosis:
Follicular dendritic cell sarcoma
Discussion
Follicular dendritic cell sarcoma is a rare neoplasm, characterized by Monda et al in 1986 7. It usually occurs as a painless, indolent mass. The median age of patients is in their fifth decade, with an age range of 17-76 years 5, and no gender preference. Patients with abdominal involvement may present with pain, but there are usually no constitutional symptoms. It may be associated with Castleman’s disease-hyaline vascular type 5, either preceding the sarcoma, or growing separately. It usually involves lymph nodes 3, predominantly cervical, axillary or mediastinal. Extranodal sites include oral cavity, spleen, liver, small intestine, pancreas, peritoneum, soft tissue, and skin. Splenic and hepatic tumors are associated with Epstein Barr virus 1, 6.
These tumors typically recur locally, with occasional distant metastases to liver or lung. In a review by Fletcher et al., it appeared that large tumor size (6 cm or more), intraabdominal location and coagulative necrosis were associated with a higher rate of recurrence, metastasis, and mortality 2.
These tumors are likely to be misdiagnosed unless one thinks of them and tests for follicular dendritic cell markers (CD21 and CD35). The main clinical and pathologic differential diagnosis, particularly within the abdomen, is a gastrointestinal stromal tumor (GIST). GIST and follicular dendritic cell sarcomas share some histologic features, including the fascicular arrangement of spindle cells, the frequent presence of epithelioid cells 4, and occasional S-100 reactivity. However, GISTs are uniformly c-kit positive and usually CD34 positive.
The differential diagnosis also includes fibroblastic reticulum cell sarcoma (vimentin+, smooth muscle actin+, desmin+, CD21-, CD35-), interdigitating dendritic cell tumor (S100+, vimentin+, CD21-, CD35-), melanoma or other sarcomas.
华夏病理/粉蓝医疗
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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
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本帖最后由 于 2007-11-11 21:39:00 编辑
FDC肉瘤其实并不十分少见，只是过去不太认识。认识免疫辅助细胞肿瘤（immune accessory cell neoplasms）的大部分成员是最近十年的事情，使淋巴组织诊断病理学的一个大进步。中华病理学杂志上已经有报道。
形态学上，FDC肉瘤还是有特点的。1）头颈部多见，2/3发生于结内，约1/3发生于结外组织；2）大体上，单发肿块，多数边界较清，体积大者有浸润，多数质地较软；3）结构上，最典型的表现是漩涡状结构，多取材多数都能见到，使人想起异位脑膜瘤或者胸腺瘤；4）细胞形态上，合体细胞型者多见，细胞梭形、圆形或上皮样；5）核形态，可由单个小而清晰的核仁，核异型性轻到中度，核分裂相可见，但不多；6）背景细胞，散在分布以成熟淋巴细胞，像点缀，B细胞为主。
有了这些特点，想到这个东东。做组化：包括CD21、CD35和CD23。要诊断这个肿瘤，其中两项阳性是必要的。之前，需用CK除外上皮性肿瘤；应用CD23是应注意这种抗体也标记某些B细胞性淋巴瘤。
不当之处，希望指正。
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