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乳头皮肤淋巴组织增生及其他淋巴组织浸润:对56例患者的回顾性临床病理学研究

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世界核心医学期刊文摘（皮肤病学分册） 2006年第4期2卷 23-24页

作者： Boud.v. L. kazakov d.v. Sima R. M. Michal 王琼 department of Pathology Lab. Spec. Diag. Medical Faculty Hospital Alej Svobody 80 304 60 Plzeˇ n Czech RepublicDr.

This study characterizes the clinicopathological spectrum of lymphoproliferations involving the breast nipple and/or areola. Morphologic, immunohistochemical, molecular-ge-netic, and clinical features of 58 specimens from 56 patients were analyzed. They were re-diagnosed as cutaneous lymphoid hyperplasia (CLH, n = 44); other benign lymphoid infiltrates (OBLI, n = 8); peripheral T-cell lymphoma,not otherwise specified (n = 1); cases with overlapping features of CLH and B-cell lymphoma (n = 3), one of them composed of spindle cells. Cutaneous lymphoid hyperplasia infiltrates were dense, composed mainly of B cells forming follicles with germinal centers (GC). Cutaneous lymphoid hyperplasia frequently showed features suggesting a malignancy as coalescing follicles with non-polarized germinal centers lacking mantle zones, and smudged infiltrates of lymphoid cells spreading into collagen (often as “ Indian files” ), smooth muscle, vessel walls, and nerve sheaths. Only two cutaneous lymphoid hyperplasias recurred; otherwise all patients are without disease (mean follow-up 62 months) . Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in five, and of T-cell receptor γ gene in two cutaneous lymphoid hyperplasias using polymerase chain reaction (PCR), but the patients fared well too. In 47% of cases Borrelia burgdorferi was detected by polymerase chain reaction and/or serology, of which one was monoclonal. We conclude that cutaneous lymphoid hyperplasia is the most common lymphoproliferation of the breast nipple, rarely recognized clinically, and often overdiagnosed histologically as lymphoma.

关键词：淋巴组织增生乳头皮肤临床病理学淋巴瘤梭形细胞生发中心滤泡博氏疏螺旋体外套层活检标本

1例肉瘤起源于皮肤和皮下透明血管型Castleman病

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世界核心医学期刊文摘（皮肤病学分册） 2005年第12期1卷 28-29页

作者： kazakov d.v. Morrisson C. Plaza J.A. M. Michal 惠海英 Sikl' s department of Pathology Charles University Medical Faculty HospitalAlej Svobody 80 304 60 Pilsen Czech Republic

We report on a case of a sarcoma arising in the hyaline- vascular variant of Castleman disease (HvCd) of the skin and subcutis. The patient was a 38- year- old man who clinically presented with a subcutaneous non- fixed cyst- like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle- cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HvCd, whereas the spindle- cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous componentwas diffusely positive for fascin, nerve growth factor receptor, and Cd34 with focal weak reactivity for Cd21 and CNA.42. Stains for Cd23, Cd31, Cd35, Cd99, ALK- 1, SMA, ASMA, desmin, factor XIIIa,AE1- AE3, EMA, bcl- 2, S- 100, Melan- A,HMB- 45, Cam 5.2, and factor vIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocyteswas detected andwe could not identify EBv or HHv- 8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth- like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.

关键词： Castleman病血管型梭形细胞核异型真皮深层淋巴滤泡双相性组织学切片邻近软组织淋巴样

多向(线性、三维囊性、圆柱性)分化的皮肤低度恶性附属器癌

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世界核心医学期刊文摘（皮肤病学分册） 2006年第11期2卷 35-36页

作者： kazakov d.v. Kutzner H. Mukensnabl P. Michal M. 李晓莉 Sikl's department of Pathology Charles University Medical Faculty Hospital Alej Svobody 80 304 60 Pilsen Czech RepublicDr.

The conjoint occurrence of follicular, sebaceous, or apocrine differentiations in a cutaneous adnexal neoplasm is a known event, more often encountered in benign neoplasms, whereas reports of cutaneous malignant adnexal tumors with bilineage or trilineage differentiation are few. A new case of a cutaneous malignant adnexal neoplasm with multidirectional differentiation is reported here. A 57-year-old woman presented with a long-standing, slowly growing, asymptomatic solitary tumor the size of a large nut in the coccygeal area,whichwas surgically excised. Ten years after the surgery, there was no evidence of recurrence or metastasis. Microscopically, the neoplasm was located in the dermis with focal extension into the subcutis. It was asymmetric, horizontally oriented, and mostly composed of small nodules that varied in shape from round and oval aggregations to elongated strands and irregular islands; the nodules were either clustered, formed a jigsaw puzzlelike pattern or were dispersed. The nodules were composed of small basaloid cells sometimes intermixed with larger cells with ample cytoplasm forming glandular structures. Rare nodules resembled elements seen in a spiradenoma by containing scattered lymphocytes and globules of hyalinized eosinophilic basal membrane material. The stroma was paucicellular, but focally it resembled that seen in perifollicular mesenchyme. Mitotic figures, including abnormal ones, were infrequent, but mild nuclear pleomorphism, nuclear crowding, and ind.v.dual cell necrosis were easily appreciable in both small basaloid cells and cells with clear cytoplasm. Perineural invasion was apparent. We classified this tumor as a well-differentiated adnexal carcinoma demonstrating combined follicular and apocrine differentiation. It differs from previously published cases of malignant adnexal tumors with multidirectional differentiation and further exemplifies the spectrum of d.v.rsity encountered in malignant proliferations with differentiation

关键词：附属器基底细胞汗腺腺瘤顶质分泌间充质腺样结构滤泡多形性新生物真皮层

眼外皮脂腺癌及皮脂腺瘤的又一尚未被认识的特殊表现:皮脂腺肿瘤类癌样表现

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世界核心医学期刊文摘（皮肤病学分册） 2005年第8期1卷 44-45页

作者： kazakov d.v. Kutzner H. Rutten A. M. Mi- chal 罗素菊 dr.Sikl' s department of Pathology Charles University Medical Faculty Hospital Alej Svobody 8O 304 60 Pilsen Czech Republic

This report emphasizes a carcinoid- like pattern, a previously unrecognized feature in cutaneous sebaceous neoplasms. We report 7 patients with sebaceous tumors in which neoplastic cells were arranged in a trabecular and ribbon- like pattern or formed rosettes/pseudorosettes. The cases included 6 men and 1 woman, with their ages at the diagnosis ranging from 43 to 87 years (median age, 59). All patients presented with a solitary lesion. Locations were the scalp (n=6) and forearm (n=1). The carcinoid- like arrangement of neoplastic cells was the sole pattern in 4 cases, and in 3 cases the so- called labyrinthine/sinusoidal and/or rippled patterns were seen in addition. Sebaceous differentiation in the form of mature sebocytes varied from almost none to approximately 10% . Although the neoplasm appeared benign architecturally, the presence of cytologic atypia qualified 2 tumors as low- grade carcinomas. Four lesions represented sebaceomas, and in 1 case microscopic delineation between a carcinoma and sebaceoma was difficult. No neuroendocrine differentiation was demonstrated immunohistochemically, histochemically, and ultrastructurally. Electron microscopic examination performed in 1 case of carcinoma revealed lipid.v.cuoles in a minority of cells. There were no membrane- bound neuroendocrine granules. Rare cells contained peculiar large helioid inclusions. We conclude that the carcinoid- like pattern is another distinctive pattern indicative of sebaceous neoplasms. This pattern seems to be closely relatedtotherippledandlabyrinthine/sinusoidalpatterns,asexemplified by our cases, in which these arrangements sometimes occurred simultaneously.

关键词：皮脂腺癌皮脂腺瘤皮脂腺肿瘤类癌皮脂腺细胞电子显微镜检查神经内分泌颗粒免疫组织化学超微结构检查大螺旋

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皮肤低分化毛母质癌肉瘤

世界核心医学期刊文摘（皮肤病学分册） 2005年第1期1卷 23-24页

作者： kazakov d.v. Kempf W. Michal M. 李晓莉冯义国 Sikl s Department of Pathology Laboratore Spec. Diagnostiky Medical Faculty Hospital Alej Svobody 80 323 18 Pilsen Czech Republic Dr.

We present an unusual case of cutaneous carcinosarcoma with the epithelial component closely resembling nodular basal cell carcinoma, and the mesenchymal component composed of cells constituting extended follicular papillae. A solitary tumor was excised in an 80-year-old man. Histologic sections revealed an ulcerated, asymmetric, poorly circumscribed neoplasm composed of epithelial cells arranged in lobules with peripheral palisading or in a cribriform pattern. The epithelial cells were darkly basophilic with scant cytoplasm and round or oval nuclei with an indistinct chromatin pattern and nucleoli. Nuclei crowding and mitotic figures were observed. Some lobules contained melanin. There were no shadow cells, sebaceous or apocrine glandular differentiation. Each epithelial nodule was surrounded by multiple rows of cells with pale vesicular nuclei and scant cytoplasm. Smaller epithelial aggregations were encircled by these cells concentrically; in larger ones these cells were aligned across a broad front resembling so-called “continuous papillae”. Additionally, numerous small follicular germ-like structures associated with papillae were seen. The cells composing “continuous papillae”showed nuclear pleomorphism, numerous mitotic figures including atypical ones, and nuclear crowding. At foci, the transition fromthe multilayered arrangement of these cells into their diffuse proliferation in the stroma was seen. There were no transitions between the epithelial and stromal component; both were intermingled as though being mutually dependent, with no areas revealing a high-grade tumor or dedifferentiation. Immunohistochemically, the epithelial cell component stained with cytokeratins. The cells of the mesenchymal component tested positive for vimentin and negative for desmin and cytokeratins. The proliferation index (Ki-67)was high in both components. Therewere also a high number of p53-positive cells in both compartments. We propose the term “low-grade trichoblastic carcin

关键词：毛母质癌肉瘤毛乳头基底细胞癌上皮结节栅栏状顶泌汗腺上皮成分高分化肿瘤间叶组织

1例罕见的由毛囊皮脂-顶泌汗腺组成的错构瘤

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世界核心医学期刊文摘（皮肤病学分册） 2006年第8期2卷 59-59页

作者： kazakov d.v. Mukensnabl P. MichalM. 吴佳纹 Sikl' s department of Pathology Charles University Medical Faculty Hospital Alej Svobody 80 304 60 PILSEN Czech Republic Dr.

Hamartoma is a neoplasm-like lesion composed of an abnormal mixture of tissues native to the area. We report on an unusual example of a hamartomatous lesion that showed an abnormal mixture of elements of the folliculosebaceous-apocrine unit and arrector pili muscle. A 13-year-old female presented with two nodules situated 2 cm apart on the lower leg that were present since birth. The patient reported some enlargement and slight pain of the otherwise asymptomatic lesions during 2 years, before they were surgically removed. The patient obviously had no skin problems afterwards; her 12-year follow-up was significant for leucopenia and nephrolithiasis. The biopsies from both lesions looked identical. Each lesion was biphasic, i.e. composed of a predominant mesenchymal component and epithelial elements. The mesenchymal part resembled a leiomyoma, or in minor areas, the constituting cells had a myofibroblastic appearance. Encased within the mesenchymal component were large infundibulocystic structures and plentiful grouped and scattered roundish solid epithelial nodules, duct-like, glandular, and tubular elements. Some of the epithelial structures were reminiscent of those seen in tubular apocrine adenoma; others resembled syringomatous elements. We interpreted this unusual lesion as a hamartoma of the folliculosebaceous -apocrine unit.

关键词：顶泌汗腺错构瘤组成皮脂毛囊白细胞减少症上皮成分皮肤病变活检标本

伴假菊形团的树突状细胞神经纤维瘤缺乏多发性神经纤维瘤2型基因1～15外显子的突变

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世界核心医学期刊文摘（皮肤病学分册） 2005年第12期1卷 24-24页

作者： kazakov d.v. vanecek T. Sima R. 李政霄 dr. Sikl' s department of Pathology Charles University Medical Faculty Hospital Alej Svobody 80 304 60 Pilsen Czech Republic

dendritic cell neurofibroma with pseudorosettes (dCNWPR) is a recently proposed.v.riant of neurofibroma with a distinctive microscopic appearance that is produced by a pseudorosette pattern formed by small, dark, lymphocyte- like cells (Type I cells) arranged concentrically around larger cells, with pale- staining vesicular nuclei and copious faintly eosinophilic cytoplasm (Type II cells). Although dCNWPR appears not to be associated with neurofibromatosis (NF), 1 patient with dCNWPR has been described and suggested to have a form of NF because of multiple skin lesions, with 2 of them being dCNWPR as confirmed histologically. The aim of this study was to find out whether the neurofibromatosis type 2 (NF2) gene is mutated in dCNWPR. Seven histologically proven cases of dCNWPR, from which a substantial amount of archival paraffin- embedded material was available, were selected for this study. Three cases have been previously reported, including the intraneural lesion, and 4 cases were newly identified. There were 3 female and 4 male patients, ranging in age from 30 to 61 years (median, 48 yrs). All patients clinically presented with a small solitary lesion that was clinically diagnosed as fibroma or neurofibroma, and none of the patients had signs of NF. Follow- up was known for 6 patients (range, 1- 5 yrs; median, 2.5 yrs) and was uneventful in each case. Microscopically, all lesions fulfilled the criteria for dCNWPR. Exons 1 to 15 of the NF2 gene were amplified by PCR using primers previously published. The amplified fragments were purified and sequenced. The obtained sequences were computer analyzed and compared with the data of the GenBank database. No mutation was identified in 5 analyzed samples from which suitable dNA had been extracted. dCNWPR appears to have no mutation in exons 1- 15 of the NF2 gene. Given the relatively small number of cases studied, it seems premature to declare that amutation of the NF2 gene is not involved in dCNWPR, as the possibility c

关键词：神经纤维瘤菊形团树突状细胞基因突变显微特征组织病理学空泡状石蜡包埋组织淋巴样细胞计算机分析

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肛门生殖器部乳腺样腺的罕见增生

世界核心医学期刊文摘（皮肤病学分册） 2006年第12期2卷 47-页

作者： kazakov d.v. Hügel H. vanecek T. Michal M. 李晓莉 Sikl's department of Pathology Charles University Medical Faculty Hospital Alej Svobody 80 30460 Pilsen Czech Republic Dr.

Anogenital mammary-like glands, although known to exist (as ectopic breast tissue) as early as 1872, have only recently been thoroughly studied and suggested to be the possible origin for various neoplastic (benign and malignant) and reactive conditions that show a striking homology with lesions in the breast. Reported herein is an unusual benign lesion involving anogenital mammary-like glands in the perineal area of a 41-year-old woman. The lesion is unique in that it does not fully fit into any of the previously recognized conditions affecting anogenital mammary-like glands nor has it an apparent well-defined mammary counterpart. Microscopically, at first glance the lesion appeared as a cluster of extended anogenital mammary-like glands of variable complexity and a lobular architecture, which probably resulted from a compact proliferation of closely situated ducts with small, round lumens (when cut transversally).When cut longitudinally, some of these tiny ducts appeared to bud from larger ductal structures, resembling so-called satellitosis in the breast and this gave a fibroadenomatous appearance to the areas. In addition, there were adenosis-like areas. This lesion extends the spectrum of changes seen in anogenital mammary-like glands.

关键词：生殖器泌尿生殖系统乳腺乳房肛门魄门

显著黑色素合成增加的黑色素瘤(动物型黑色素瘤)超微结构研究报道

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世界核心医学期刊文摘（皮肤病学分册） 2005年第1期1卷 22-22页

作者： kazakov d.v. Rüutten A. Kempf W. Michal M. 罗素菊冯义国 Sikl's department of Pathology Medical Faculty Hospital Alej Svobody 80 304 60 Pilsen Czech Republic Dr.

Melanoma with prominent pigment synthesis or animal-type melanoma (ATM) is a very rare type of melanoma. Its histogenesis has not been elucidated and ultrastructural features have not been described in human beings. We present an additional case of ATM in a 28-year-old woman with positive sentinel node biopsy and provide the results of electron microscopic studies. Histopathologically, the skin lesion was composed of heavily pigmented neoplastic cells mostly arranged as large sheets, focally also in a nodular growth pattern. After bleaching, the neoplastic cells demonstrated round nuclei with 1 or rarely 2 conspicuous nucleoli and a prominent nuclear membrane and abundant, gray, slate-like cytoplasm. Some cells demonstrated round cytoplasmic inclusions. There was no nuclear pleomorphism, and only a few mitotic figures could be found after extensive search. Multiple areas of necrosis en masse of tumor cells were seen. The lymph node biopsy revealed a complete effacement of the lymph node architecture by the extensive proliferation of hyperpigmented cells in the parenchyma. Immunohistochemically, the same pattern of staining was seen on the bleached and unbleached slides both in the skin and in the lymph node. The neoplastic cells stained positively with MiTF (nuclei), NSE, NKI/C3, tyrosinase (weak), p53, and Cd68. S-100 protein, HMB45, Melan A, Mac367, and lysozyme reacted ngatively. Occasional cells (d with MIB-1. Ultrastructural studies revealed that the neoplastic cells possessed a large, indented nucleus with a prominent nuclear membrane, a single (para) centrally located nucleolus, and peripherally marginated chromatin. The cytoplasm was abundant and contained numerous single melanosomes and rare compound melanosomes. The melanosomes were in stages II to Iv of maturation, with a marked predominance of stage II and stage III melanosomes. There was a high number of aberrant melanosomes with a wide variety of configurations. Melanophages were a minor co

关键词：黑色素瘤黑色素合成超微结构研究黑素瘤异形性前哨淋巴结活检电子显微镜检查核分裂相包涵体组织发生