巨大毛囊皮脂腺囊性错构瘤

报告1例巨大毛囊皮脂腺囊性错构瘤。患者男,15岁。右臀部丘疹,结节15年。体格检查示右臀部多个大小不一的丘疹及结节、部分融合成硬斑块。组织病理检查示毛囊扩张畸形,其上端为毛囊漏斗部扩张,形成囊性结构,下端为增生的皮脂腺,周围胶原增生硬化,且在真皮浅层及中部可见散在成熟的脂肪组织。诊断：巨大毛囊皮脂腺囊性错构瘤。     

Giant folliculosebaceous cystic hamartoma

We report a case of a giant cystic cutaneous adnexal hamartoma which showed fibrofollicular units embedded in the stroma with aberrant fat tissue. There were many sebaaeous lobules in the fibrofollicular units, and its histologic features were consistent with conventional folliculosebaceous cystic hamartoma. Given the unique clinical history and appearance, this lesion is considered to be a congenital variant.     

毛囊皮脂腺囊性错构瘤研究进展

临床上,毛囊皮脂腺囊性错构瘤比较少见,缺乏典型特征,是一种不对称,好发于面部的肤色丘疹或结节。病理学上是以毛囊皮脂腺组织和间叶细胞成分增生为主的皮肤附属器错构瘤,主要与皮脂腺毛囊瘤、皮样囊肿相鉴别。免疫组化结果与瘤体内上皮或非上皮成分有关,无特异性。概述毛囊皮脂腺囊性错构瘤的临床表现、诊断标准、免疫组化及治疗方面的研究进展。     

Two cases of folliculosebaceous cystic hamartoma

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units associated with mesenchymal elements. Two cases of FSCH with typical histopathological features are reported. Patient 1 was a 60-year-old man presented with a normal skin-coloured asymptomatic nodule on his scalp. Patient 2 was a 70-year-old man with an asymptomatic nodule on his right auricle that had persisted for the previous 15 years. In all, 34 cases of FSCH have been reported in the English literature. Clinically, the lesions are asymptomatic, usually rubbery to firm in consistency, and usually occur on or above the neck (> 90%). Most lesions do not exceed 25 mm in diameter (> 90%). Histopathologically, FSCH shares several similar features to sebaceous trichofolliculoma, but it is usually possible to differentiate these two tumours.     

毛囊皮脂腺囊性错构瘤一例

患者男,23岁.因左颈部斑块3～4年,于2008年6月30日就诊.患者自述左侧颈部3～4年前出现小丘疹,无自觉症状.逐渐增大增多,丘疹融合成斑块.患者平素身体健康,未经任何治疗.     

巨大毛囊皮脂腺囊性错构瘤

报告1例巨大毛囊皮脂腺囊性错构瘤。患者男,29岁,左眼眶外侧及颞部出现红色丘疹20年,增大为结节和斑块8年。皮肤科检查:左眼眶外侧暗红色结节,部分与额部暗红色斑块相连,结节和斑块表面见多处凹陷,质地软,无触痛,无内容物挤出。左眼眶外侧结节组织病理检查:真皮中部可见多个畸形的毛囊结构,毛囊漏斗部扩张,与增生的皮脂腺小叶相连,周围有增生硬化的胶原,真皮浅中层可见增生的毛细血管。诊断:巨大毛囊皮脂腺囊性错构瘤。     

A clinicopathologic study of folliculosebaceous cystic hamartoma

Folliculosebaceous cystic hamartoma (FSCH) is a distinctive cutaneous hamartoma of follicular, sebaceous, and mesenchymal components. Only 70 cases of FSCH have been reported in the literature since the original report of 5 cases in 1991. There has been little information reported about the clinicopathologic characteristics of FSCH. We summarize the clinicopathologic features of 153 cases of FSCH that were diagnosed histopathologically at Sapporo Institute for Dermatopathology. The 153 cases of FSCH comprised 92 male and 61 female patients. The typical clinical presentation of FSCH revealed solitary and skin-colored, protruding papules or nodules measuring several millimeters in diameter on the face, especially on the nose, of middle-aged or older persons. These cases fulfilled the common denominators for the histopathologic diagnosis of FSCH as Kimura et al reported. Seven of 153 cases (4.6%) were accompanied by distinctive features of Miescher-type melanocytic nevi. All 7 cases showed lesions on face, especially on or around the nose. We consider that Miescher-type melanocytic nevi play a significant role in the pathogenesis of FSCH, at least in some cases.     

先天巨大毛囊皮脂腺囊性错构瘤一例

患儿男,37 d龄,因左下颌肿块37 d就诊。患儿出生时左下颌可见鸡蛋大小红色肿瘤,未行诊疗,后肿瘤逐渐增大,14 d时在我科门诊就诊,行磁共振及小部分皮损组织病理检查,提示皮肤错构瘤,因年幼未予特殊治疗。肿瘤体积逐渐增大,成倍增长,影响颈部活动,再次就诊。患儿系足月剖宫产,母乳喂养,生长发育同正常同龄儿,家族中无类似病史……     

Giant, congenital folliculosebaceous cystic hamartoma: a case against a pathogenetic relationship with trichofolliculoma

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma consisting of dilated folliculosebaceous units invested in mesenchymal elements. These solitary lesions typically affect adults, have a predilection for the central face or scalp, and remain less than 1.5 cm in greatest dimension. Although giant FSCH variants have been documented, the congenital presentation of a giant FSCH stands in contrast to earlier views of its histogenetic relationship with trichofolliculoma. We describe a giant pedunculated FSCH arising from the posterior neck of a 9-month-old Hispanic male and discuss various aspects of this highly unusual case.     

Clinicopathological and immnuohistochemical findings in a series of folliculosebaceous cystic hamartoma

Background:  Folliculo-sebaceous cystic hamartoma (FSCH) is an uncommon skin condition presenting as a slow-growing papulo-nodular lesion, in or around the nose. Most cases are not clinically suspected and only histopathological examination allows the diagnosis. Pathological features include a dermal-located infundibulo-cystic structure with sebaceous glands radiating around, a stromal component encircling the epithelial structures, with clefts between the lesional epithelial and stromal parts, as well as between this and the adjacent dermis.
Results:  We report eight patients with the diagnosis of FSCH (5 females and 3 males), with ages ranging from 35 to 77 years. Most cases (5 out of 8) were located in or around the nose and sizes were comprised between 0.6 and 1.2 cm. Lesions had grown for long periods of time, up to ten years in one case. Immunohistochemistry showed staining for p63 in the epithelial component of all lesions, while CD10 was only present in some sebocytes. CD34 and Factor XIIIa positive cells were present in the lesional stroma. Staining for androgen and alpha-estrogen receptors was also usually noticed.
Conclusions:  FCSH is a hamartomatous skin lesion, clinically indistinct but with well-defined histopathological features. Immunohistochemistry shows a profile very close to normal sebaceous glands.     

A case of genital folliculosebaceous cystic hamartoma with an unique aggregated manifestation

Folliculosebaceous cystic hamartoma (FCH) is a recently recognized cutaneous hamartoma composed of follicular, sebaceous and mesenchymal components, and usually occurring on the head and neck. We describe herein a case of FCH with an unique aggregated manifestation in a genital lesion. The patient was a 40-year-old woman with a genital lesion composed of a pedunculated nodule, a dome-shaped nodule and a subcutaneous nodule, measuring 5 cm in the greatest dimension. The largest, pedunculated nodule was histologically composed of an infundibulo-cystic structure with follicular, sebaceous and mesenchymal elements accompanied by cystic structures of various sizes lined by stratified squamous epithelium and follicular germinative cells suggesting follicular cysts. The dome-shaped nodule consisted of anastomosing strands of epithelial cells with follicular components. The subcutaneous nodule had two components, an infundibulo-cystic structure and a cyst lined by squamous epithelium. In our case, the unusual clinical feature of large and multiple nodules was due to the presence of several prominent hamartomatous cystic structures with FCH. This is the third case of giant FCH. The clinical presentation and location of giant FCH is unusual.     

Giant nevus lipomatosus superficialis with multiple folliculosebaceous cystic hamartomas and dermoid cysts

Nevus lipomatosus superficialis (NLS) is a rare benign condition characterized by papules and nodules usually in the pelvic and sacral areas, with ectopic mature adipose tissue reaching the superficial dermis. We report the case of a 47-year-old female with large NLS in the sacral region that had been present since birth and, after an asymptomatic course for most of the time, became associated with episodes of mild pain that prompted the patient to look for medical help. After a complete resection of the cerebriform plaque, measuring 23 cm in diameter, histopathological examination revealed typical NLS architecture in as yet undescribed association with multiple folliculosebaceous cystic hamartomas (FCHs) and dermoid cysts.     

Folliculo-sebaceous cystic hamartoma.

Case 1 A 6‐month‐old boy presented to our clinic with a scalp lesion dating since birth ( Fig. 1 ). The lesion was in the form of an erythematous, dome‐shaped nodule, measuring around 1.5 cm in diameter and having a slightly irregular surface. The lesion was nonitchy, nontender, and showed limited mobility. Our clinical differential diagnosis included: meningocele, hemangioma, dermoid cyst, and apocrine nevus. A computed tomography (CT) scan was performed to exclude possible intracranial connection, and the lesion was surgically excised.

Figure 1 Open in figure viewer PowerPoint Case 1: erythematous nodule on the occipital area of a 6‐month‐old boy. The lesion was clinically suggestive of apocrine nevus, dermoid cyst, or meningocele     

Folliculosebaceous cystic hamartoma of the ear and periauricular skin

Folliculosebaceous cystic hamartoma (FSCH) is a rare, recently recognized cutaneous hamartoma comprised of follicular, sebaceous and mesenchymal elements. Fewer than 30 cases have been reported to date. The authors present two additional cases of FSCH.     

Folliculosebaceous cystic hamartoma with hair shaft fragments

We describe herein a 57-year-old Japanese male with a folliculosebaceous cystic hamartoma (FCH) presenting several hair shaft fragments in the cyst. Clinically, a skin-colored, dome-shaped, hard nodule, 5 x 4 mm in diameter was observed on the antitragus of his left ear. Histological examination revealed a large cystic structure surrounded by multiple mature sebaceous lobules and rudimentary hair follicules in the middle of the dermis. The cystic structure contained laminated orthokeratotic keratin and several hair shaft fragments. The fibrous stroma invaded the sebaceous lobules and obvious clefts were seen around the folliculosebaceous units located in the dermis. These features are characteristic histological findings of FCH. To our knowledge, this case is unusual with the presence of hair shaft fragments within the cystic structure. In addition, we discuss the histopathological findings of FCH and also review other cases of FCH in the literature.