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2006年5月广东省病理学会读片会内容和结果(二)
 


更新时间:2007-04-04 11:18:45 

 

中山大学肿瘤防治中心 病理科

病例一 病理号:349443
病史摘要:
男性,41岁
因涕血、鼻塞伴皮疹,睾丸肿大2月余入院。
体检:躯干和上肢散在分布多个红疹,触之结节状,浅表淋巴结无肿大,扁桃体不大,肝脾未触及,右睾丸明显肿大,质硬,大小约8*9*9CM,无明显触痛。

MR示:双侧鼻窦炎。
CT示:左鼻窦炎,左鼻息肉,左上肺占位病变,肝内低密度结节灶。

临床诊断:睾丸癌?

大体检查:送检睾丸及睾丸旁肿 物大小一共为13*9*5CM,其中睾丸大小为6.5*4*3CM,睾丸旁肿物大小为9*7*4CM,切面淡黄色,质脆。
 


镜下检查:睾丸结构已被破坏,尚可见少量残存的曲细精管,瘤细胞小至中等大小,圆形、卵圆或不规则形,胞浆中等量,核分裂像易见,瘤细胞弥漫浸润于曲细精管间,可见浸润血管壁,但未见明显坏死。

 


免疫组化:LCA(+),cCD3(+),UCHL1(+),CD43(+),CD56(+),GranzymeB(+),TIA-1(+),Perf(+) CD79a(-),CD20(-),CD5(-),CD10(-),CYClinD1(-),TdT(-),MPO(-),CD34(-),CK(-)。
原位杂交:EBERS(+)。

病理诊断:
(右侧睾丸) 结外NK / T细胞淋巴瘤,鼻型

诊断依据:
1、瘤细胞弥漫浸润于曲细精管之间,瘤细胞浸润及破坏血管壁。
2、免疫组化:LCA(+),cCD3(+),UCHL1(+),CD43(+),TIA-1(+),颗粒酶B(+),CD56(+),Perf(+)
3、原位杂交:EBERS(+)。

鉴别诊断:
1、精原细胞瘤;
2、未分化癌;
3、PNET;
4、胚胎性横纹肌肉瘤。

Ko YH, Cho EY,et al. NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status. Histopathology. 2004 May;44(5):480-9.
★The primary sites of 51 cases of cCD3+ TIA+, CD56+ extranodal lymphoma:
soft tissue (n = 10), the gastrointestinal (GI) tract (n = 13), the skin (n = 15), upper aerodigestive tract excluding nasal and nasopharyngeal regions (n = 11), the testis (n = 1), and parotid gland (n = 1).
★TCR gene rearrangement was detected in seven of 47 cases examined (16%).
★EBV was positive in 39 of 51 cases (76%).
★The positive rate of EBV was higher in tumours of soft tissue (80%), GI tract (92%), and skin (80%), and lowest in the upper aerodigestive tract excluding the nasal and nasopharyngeal region (50%).

Kurpis M, Diller AB, Calafat P[Extranodal NK/T cell lymphoma of the nasal type] Rev Fac Cien Med Univ Nac Cordoba. 2002;59(1):117-20.
★The vast majority of lymphoma occurring in the testis are diffuse large B-cell Lymphoma.
★We report on a case of natural killer/T cell lymphoma in a forty four year old male, with bilateral testicular and cutaneous involvement with a highly aggressive course.
★The tumor cells were positive for both CD56 and Epstein Barr Virus, encoded EBER molecules, and showed focal angiocentric growth thus fullfilling the criteria for NK/T cell lymphoma of nasal type.
★Dermatopathologists and uropathologists should be aware of this rare entity which may only be diagnosed after extensive inmunohistochemical studies.

Kim YB, Chang SK, Yang WI, Primary NK/T cell lymphoma of the testis. A case report and review of the literature. Acta Haematol. 2003;109(2):95-100.
★We report a case of aggressive 'nasal type' natural killer (NK)/T cell lymphoma initially presenting as a testicular tumor in a Korean man, which quickly took a fatal course by widespread dissemination.
★The Epstein-Barr virus genome was detected by in situ hybridization.

Liang R. Diagnosis and management of primary nasal lymphoma of T-cell or NK-cell origin. Clin Lymphoma. 2000 Jun;1(1):33-7;
★On immunophenotyping, the diagnostic features are surface CD3(-), cytoplasmic CD3 epsilon(+), and CD56(+).
★For the majority of the cases, T-cell receptor gene rearrangement is absent, confirming an NK-cell origin of the tumor cells.
★Clonal proliferation of Epstein-Barr virus (EBV) is usually present in the tumor cells and is a useful diagnostic marker.
★The tumor may also disseminate to skin, gastrointestinal tract, and the testis at the time of progression.

Chan JK, Sin VC, Wong KF, Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. 1997 Jun 15;89(12):4501-13.
★ The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen.
★The characteristic immunophenotype was CD2+ CD3/Leu4- CD3epsilon+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV).


病例二
?
病理号:359453

病史摘要:
女性,43岁。?
发现左上臂肿物4年余,增大1月余。4年前发现外阴肿物于当地医院切除,病理示“巨大淋巴结增生”。1年前又因左侧乳腺肿物于当地医院切除,病理示“肉芽肿性炎症伴淋巴组织增生活跃”。
?
临床诊断:左上臂肿物性质待查。

大体检查:送检结节样肿物大小为5*3*2CM,包膜完整,切面灰白,质中。


 


镜下检查:淋巴结的大部分已被破坏,有少量淋巴滤泡残留,滤泡间区增大增宽,细胞成份较杂,可见浆细胞,嗜酸性粒细胞等,小血管增生明显,其中可见多量小至中等大小的异型淋巴细胞弥漫增生,核膜不规则,有曲核,胞浆少至中等量,核分裂像易见。

 


免疫组化结果:
CD3(+),UCHL1(+),CD5(+),
Ki67 20%(+) 。

L26(-),CyclinD1(-),CD10(-),CD23(-),CD30(-),TIA-1(-),
颗粒酶B(-),Perf(-),CD56(-),ALK(-)。

诊断:(左上臂淋巴结)外周T细胞性淋巴瘤,非特殊性。

诊断依据:
1、淋巴结的结构大部分被破坏,T区异型淋巴细胞增生,小血管明显增生;
2、免疫组化:CD3(+),UCHL1(+),CD5(+),Ki67 20%(+),CD21(-)。

鉴别诊断:

1、淋巴组织反应性增生;
2、 HD 。


病例三
?
病理号:363273


?病史摘要:

右膝内侧皮肤肿物术后5年,复发3周

体检:右膝内侧皮肤结节样肿物直径为2CM,局部皮肤破溃。
?
临床诊断:右膝肿物性质待查。

大体标本:送检皮肤组织大小为4*2*1CM,皮肤见结节2*2*1CM,局部皮肤破溃,切面灰黄,质软,与周围组织分界尚清。

 


镜下检查:肿物表面溃疡形成,溃疡底部至真皮深层均可见异型细胞弥漫浸润,细胞体积较大,胞浆丰富,核大、间变明显,呈圆形、卵圆形或肾形,核膜清楚,核仁明显,1个或数个,可见多核花环状、马蹄形及R-S细胞样瘤细胞,核分裂像多见,瘤细胞间可见多量中性粒细胞和组织细胞等浸润,血管增生明显。


 


免疫组化结果:
LCA(+),CD30(+),EMA(+),Vim(+),CD3(+),UCHL1(+),Perf(+),TIA-1(+),颗粒酶B(+)

CD79a(-),L26(-),CK(-),S100(-),HMB45(-),HHF35(-),Des(-),Myo(-),CD34(-),CD31(-) ,ALK(-) 。

诊断:(右膝内侧)原发性皮肤间变性大细胞淋巴瘤。

诊断依据:
1、病灶单个,呈结节状;
2、临床进展慢(惰性);
3、瘤细胞呈片状分布,浸润至真皮层。

4、免疫组化:
LCA(+),CD30(+),CD3(+),UCHL1(+),Perf(+),颗粒酶B(+),TIA-1(+) 。

鉴别诊断:
1、淋巴瘤样丘疹病;
2、(系统性)间变性大细胞淋巴瘤,侵犯皮肤;
不支持点:A、病程进展慢;
B、全身浅表淋巴结无累及。

3、蕈样霉菌病;
4、恶性黑色素瘤;
5、横纹肌肉瘤;
6、未分化癌。

Slater DN. The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants. Br J Dermatol. 2005 Nov;153(5):874-80.
★Cutaneous T-cell lymphomas include :
(1) Folliculotropic mycosis fungoides,
(2) Primary cutaneous CD30+ lymphoproliferative disorders
(2.1) Primary cutaneous anaplastic large cell lymphoma
(2.2) Lymphomatoid papulosis
(2.3) Borderline lesions)
(3) Subcutaneous panniculitis-like T-cell lymphoma.
(4) Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma,
(5) Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
(6) Cutaneous gamma/delta T-cell lymphoma
(7) Unspecified primary cutaneous peripheral T-cell lymphoma.


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