| 图片： | |
|---|---|
| 名称： | |
| 描述： | |
- B1278Vulvar anaplastic large T cell lymphoma ALK-1 negative (cqz1)
图1
图2
图3
图4
| 姓 名： | ××× | 性别： | F | 年龄： | 65-70 |
| 标本名称： | Vulvar lesion | ||||
| 简要病史： | |||||
| 肉眼检查： | |||||
Just signed a case two weeks ago and like to share it with all friends here.
Vulvar mass 5 cm. No previous malignant history.
Fig Key:
Fig 1. 2x
Fig 2. 20x juction
Fig 3. 20x tumor
Fig 4. 40x tumor
Tumor shows similar morphology in different areas.
Please give your differential dx and the requirement for IHC stains if it is your case. What is your favor diagnosis based on the H&E slides?
I will have few weeks vocation and will poster the immunostain photos or results after I come back.
Thnaks,
标签：ATCL ALK阴性
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本帖最后由 于 2009-02-25 09:47:00 编辑
×参考诊断
ATCL，ALK阴性
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wangdingding 离线
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本帖最后由 于 2008-11-14 20:10:00 编辑
| 以下是引用abin在2008-11-13 20:58:00的发言： 谢谢Dr.cqzhao纠正。我猜fellow是住院医师，fellowship training 是住院医师培训。说不定又猜错了，哈哈。期待Dr.cqzhao有空给我们谈谈这方面的中美差别。 |
住院医师=residents
Fellow: One year of subspecielty training after the residency training (now pathology 4 years), such as gynecologic pathology, hemato pathology, GI pathology et al. They are not attending pathologists. It is not a job. I am happy to talk about these in future on tele.
abin译：
Fellow: 住院医生培训之后的一年亚专业培训（现在病理专业的培训是4年），如妇科病理，血液病理，消化病理等。他们不是病理主治医生（？attending pathologists？汗，又要猜了），也不是一门职业。我很乐意以后远程谈谈这些。
ALCL, ALK 阴性
¢ 作为一个暂定的实体包括在最近的WHO分类中。
l “CD30＋的T细胞肿瘤，与ALC,ALK＋相比，在形态学上没有可重复地用于鉴别的根据”
l 与ALK＋ALCL相比，具有较大的中位年龄和更具有侵袭性的临床过程。
¢ 认为与外周T细胞淋巴瘤（NOS）是不同的，但是这种区别总是很不明显。
¢ 发病高峰是40－65岁。
¢ 男性多见，男女比例1.5:1。
¢ 累及淋巴结和淋巴结外部位，但通常比ALCL,ALK＋更少见。
¢ 肿瘤细胞较大并且更具有多形性。
¢ 一半以上的病例表达一个或者更多T细胞标记；CD4+/CD8-
¢ CD2和CD3经常是阳性。
¢ EMA很少阳性。
¢ 大多数表达细胞毒相关的标记。
¢ 大多数表现为T细胞受体克隆性基因重排。
¢ 通常没有细胞遗传学的变异。
ALCL, ALK 阴性鉴别诊断
¢ 鉴别诊断
l 原发皮肤的ALCL
l 其他原发皮肤CD30＋淋巴组织增生性疾病
• 但是对于定义，淋巴瘤样丘疹病（LyP）仅限于皮肤。
l 外周T细胞淋巴瘤，NOS
¢ 原发皮肤 ALCL
l 在诊断的时候，疾病仅限于皮肤。
l 可以发生皮肤外的播散，主要是在局部的淋巴结。
l 主要发生在成年人和老年人。
l T细胞的免疫表型CD2,CD5和/或CD3有不同程度的丢失；大于75％的细胞表达CD30。
l 大多数病例EMA和ALK是阴性的。
l 整体五年生存率为90％。
¢ 外周T细胞淋巴瘤, NOS
l 可以发生在淋巴瘤或淋巴结外；皮肤和胃肠道也通常发生。
l 异常的背景经常是大淋巴细胞混有炎细胞。
l 没有淋巴结窦内浸润模式。
l T细胞免疫表型；CD4+/CD8-
l CD2，CD3经常是阳性的。
l CD30可以有表达，但是通常是强度不等，不是那种很强的、一致的细胞膜阳性的模式。
结论
¢ 发生在外阴的淋巴瘤，原发的或者继发的
¢ 最常见的类型是弥漫大B细胞淋巴瘤。
¢ 常发生于老年女性。
¢ ALCL, 系统性或原发皮肤类型
l ALCL，ALK阴性，新WHO分类中暂定实体。
l 证据显示ALK+ ALCL, ALK- ALCL, 和 PTCL, NOS 是不同的实体。
¢ 外阴肿块的鉴别诊断应该包括淋巴瘤。
以上是我对abin老师没有翻译的部分做了简单翻译，可能不是特别理想，请各位网友见谅。
Thank abin for the excellent translation.
Fellow does not mean colleaque. In the US you need four years of pathology residency training after the medical school. Most people will do one or two fellowship training in some subspecieties. It needs one year of fellow training. So fellow means one subspecilty training after residency training. Pathology fellows include many subspecieties, GI, gynecology, breast, derm, hematology, GU, ENT et al.
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本帖最后由 于 2008-11-12 23:00:00 编辑
| 以下是引用cqzhao在2008-11-12 11:30:00的发言并翻译：
上述淋巴结为腹股沟淋巴结。 病例由我们的妇科病理同事在科室讨论会上提供。我把她的一些总结和大家分享。 ¢ 累及女性生殖道的原发性结外淋巴瘤：0.2 to 1.1% l 宫颈和卵巢最常见，其次是宫体和阴道，外阴和输卵管最少 ¢ 累及女性生殖道的继发性结外淋巴瘤： 7 to 30% 例 l 卵巢最常见，其次是子宫
¢ 外阴淋巴瘤患者倾向于比其它部位发生者年龄更大，平均60岁 ¢ DLBCL是最常见的累及女性生殖道的淋巴瘤（输卵管除外） ¢ 外阴DLBCL最常见，其次为滤泡性淋巴瘤
ALCL, ALK Positive
¢ 约占成人NHL的3% l 10-20%为儿童 ¢ ALK阳性最常见于30岁之前，多为男性 ¢ 通常累犯LN和结外 l 皮肤最常见(21%), 骨(17%), 软组织(17%), 肺(11%), 肝 (8%). ¢ HE染色观察到10%累犯骨髓 l 运用IHC染色CD30, EMA和/或ALK后增加到30% ¢ 宽广的形态学谱系 ¢ 所有病例均显示细胞核偏心、马蹄形或肾形=特征性细胞 ¢ 可出现多核，花环样 ¢ 一般比大多数淋巴瘤更富于胞浆 ¢ CD30呈膜阳性 ¢ t(2;5)-NPM/ALK 易位是最常见的遗传学改变 l 显示核和胞浆着色 l ALK蛋白融合，通常与NPM形成跨膜蛋白 ¢ ALK表达实际上是ALCL的特征 ¢ 不见于其他人类肿瘤 l 罕见DLBCL l 罕见横肉 l 炎性肌纤母细胞肿瘤 ¢ 大部分EMA阳性
¢ 绝大多数表达一种以上的T-cell抗原; CD4+/CD8-. ¢ CD45表达不一 ¢ 大多数表达细胞毒性标记物：TIA1, 粒酶B, and/or 穿孔素. ¢ 90%呈T-cell受体克隆性重排 ¢ 通常无EBV
ALCL, ALK Negative
¢ Included as provisional entity in most recent WHO. l “CD30+ T-cell neoplasm not reproducibly distinguishable on morphological grounds from ALCL, ALK+”. l Older median age and more aggressive clinical course than ALK+ ALCL. ¢ Considered distinct from peripheral T-cell lymphoma, NOS, but distinction is not always straightforward. ¢ Peak incidence 40-65 years. ¢ Male predominance, 1.5:1. ¢ Involves lymph nodes and extranodal sites, but less commonly than ALCL, ALK+. ¢ Tumor cells larger and more pleomorphic. ¢ More than half of cases express one or more T-cell markers; CD4+/CD8-. ¢ More likely to be positive for CD2 and CD3. ¢ Fewer positive for EMA. ¢ Majority show cytotoxic-associated markers. ¢ Most show clonal rearrangement of T-cell receptor. ¢ No recurrent cytogenetic abnormalities.
ALCL, ALK Negative DDx ¢ Differential diagnosis l Primary cutaneous ALCL l Other primary cutaneous CD30+ lymphoproliferative disorders • But lymphomatoid papulosis (LyP) is, by definition, limited to skin l Peripheral T-cell lymphoma, NOS ¢ Primary cutaneous ALCL l Disease is limited to skin at time of diagnosis. l Extracutaneous dissemination may occur, mainly to regional lymph nodes. l Predominantly in adults and elderly. l T-cell phenotype with variable loss of CD2, CD5 and/or CD3; CD30 expression >75% cells. l Most cases negative for EMA and ALK. l Overall 5-year survival rate of 90%. ¢ Peripheral T-cell lymphoma, NOS l May be nodal or extranodal; skin and GI tract most common. l Abnormal medium to large lymphocytes often admixed with inflammatory cells. l Sinus pattern of LN infiltration is absent. l T-cell phenotype; CD4+/CD8-. l More likely to be positive for CD2, CD3. l CD30 may be seen, but usually with variable intensity, and not in strong, homogeneous, membranous pattern. Conclusions ¢ Lymphoma, primary or secondary, is rare in the vulva. ¢ Most common type is DLBCL. ¢ Tend to occur in older women. ¢ ALCL, systemic and primary cutaneous types. l ALCL, ALK-negative provisional entity in new WHO. l Evidence that ALK+ ALCL, ALK- ALCL, and PTCL, NOS are distinct entities. ¢ Lymphoma should be included in the differential diagnosis of vulvar mass.
我们在文献中没有搜索到外阴 ALCL，我要同事写一篇病例报道。 现在你已经看到这个病例的全部内容。
谢谢讨论和浏览。 接着，是子宫 |
sorry!
翻译这些对我来说太难了，因为我不懂淋巴瘤。其余的内容要请其他朋友帮助一下。谢谢
abin
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。
Above lymph node is inguinal LN.
This case was present by our GYN fellow in the dept conference. I share some of her summary with you.
¢ Primary extranodal lymphoma involving the female genital tract ranges from 0.2 to 1.1%.
l Cervix and ovaries most frequent, then uterine corpus and vagina, with vulva and fallopian tubes least common.
¢ Disseminated lymphoma secondarily involves the female genital tract in 7 to 30% of cases.
l Ovary most commonly, then uterus.
¢ Patients with vulvar lymphoma tend to be older than patients with lymphoma at other sites, mean 60 years.
¢ DLBCL is the most common lymphoma affecting the female genital tract (except in fallopian tube).
¢ In vulva, DLBCL is most frequent, with follicular lymphoma next.
ALCL, ALK Positive
¢ Accounts for approximately 3% of adult non-Hodgkin lymphomas.
l 10-20% of childhood.
¢ ALK-positive is most frequent first three decades of life, and shows male predominance.
¢ Usually involves LN and extranodal sites.
l Most frequent skin (21%), bone (17%), soft tissue (17%), lung (11%), and liver (8%).
¢ Bone marrow involvement 10% with H&E.
l Increased to 30% when immunostains for CD30, EMA and/or ALK applied.
¢ Broad morphologic spectrum.
¢ All show cells with eccentric, horseshoe- or kidney-shaped nuclei = “hallmark cells”.
¢ Multiple nuclei may occur in a wreath-like pattern.
¢ In general, more abundant cytoplasm than most lymphomas.
¢ CD30 positive in a membranous pattern.
¢ Translocation t(2;5)-NPM/ALK is most frequent genetic alteration.
l Shows nuclear and cytoplasmic staining.
l Fusion of ALK protein, usually transmembrane with NPM, nuclear transport protein.
¢ ALK expression virtually specific for ALCL.
¢ Absent from most other human neoplasms.
l Rare DLBCL.
l Rare cases of rhabdomyosarcoma.
l Inflammatory myofibroblastic tumors.
¢ Majority positive for EMA.
¢ Vast majority express one or more T-cell antigens; CD4+/CD8-.
¢ Variably positive for CD45.
¢ Most positive for cytotoxic-associated antigens TIA1, granzyme B, and/or perforin.
¢ 90% show clonal rearrangement of T-cell receptor.
¢ EBV is always absent.
ALCL, ALK Negative
¢ Included as provisional entity in most recent WHO.
l “CD30+ T-cell neoplasm not reproducibly distinguishable on morphological grounds from ALCL, ALK+”.
l Older median age and more aggressive clinical course than ALK+ ALCL.
¢ Considered distinct from peripheral T-cell lymphoma, NOS, but distinction is not always straightforward.
¢ Peak incidence 40-65 years.
¢ Male predominance, 1.5:1.
¢ Involves lymph nodes and extranodal sites, but less commonly than ALCL, ALK+.
¢ Tumor cells larger and more pleomorphic.
¢ More than half of cases express one or more T-cell markers; CD4+/CD8-.
¢ More likely to be positive for CD2 and CD3.
¢ Fewer positive for EMA.
¢ Majority show cytotoxic-associated markers.
¢ Most show clonal rearrangement of T-cell receptor.
¢ No recurrent cytogenetic abnormalities.
ALCL, ALK Negative DDx
¢ Differential diagnosis
l Primary cutaneous ALCL
l Other primary cutaneous CD30+ lymphoproliferative disorders
• But lymphomatoid papulosis (LyP) is, by definition, limited to skin
l Peripheral T-cell lymphoma, NOS
¢ Primary cutaneous ALCL
l Disease is limited to skin at time of diagnosis.
l Extracutaneous dissemination may occur, mainly to regional lymph nodes.
l Predominantly in adults and elderly.
l T-cell phenotype with variable loss of CD2, CD5 and/or CD3; CD30 expression >75% cells.
l Most cases negative for EMA and ALK.
l Overall 5-year survival rate of 90%.
¢ Peripheral T-cell lymphoma, NOS
l May be nodal or extranodal; skin and GI tract most common.
l Abnormal medium to large lymphocytes often admixed with inflammatory cells.
l Sinus pattern of LN infiltration is absent.
l T-cell phenotype; CD4+/CD8-.
l More likely to be positive for CD2, CD3.
l CD30 may be seen, but usually with variable intensity, and not in strong, homogeneous, membranous pattern.
Conclusions
¢ Lymphoma, primary or secondary, is rare in the vulva.
¢ Most common type is DLBCL.
¢ Tend to occur in older women.
¢ ALCL, systemic and primary cutaneous types.
l ALCL, ALK-negative provisional entity in new WHO.
l Evidence that ALK+ ALCL, ALK- ALCL, and PTCL, NOS are distinct entities.
¢ Lymphoma should be included in the differential diagnosis of vulvar mass.
We did not find valvar ALCL report in literature serach. I asked the fellow to write a case report.
Now you see all of the case already.
Thank for discussion and reviewing of this case.
, then uterus
