毛母质瘤336例临床回顾性分析

目的回顾性分析336例毛母质瘤的临床和组织病理资料,提高对该病的认识。方法对我院336例组织病理确诊为毛母质瘤患者的临床表现和组织病理资料进行回顾性分析。结果两性发病率无差异,青年期为发病的就诊高峰,头面部、颈部为好发部位。临床表现多样,患者初诊科室较多,临床误诊率高。结论该病临床表现多样,误诊率高,临床医师需提高对该病的认识。     

Malignant pilomatricoma

A 47-year-old woman presented with a nodular lesion in the left retroauricular area. The lesion was enucleated and a diagnosis of malignant pilomatricoma was made. This was treated by wide excision and no residual tumour was found. The patient remained disease-free at the 18 months follow-up.     

An investigation of cytokeratin expression in skin epithelial cysts and some uncommon types of cystic tumours using chain-specific antibodies

Summary The differentiation state of skin epithelial cysts and some uncommon types of epithelial skin tumours was investigated by immunohistochemical staining, mainly using cytokeratin (CK) polypeptide-specific monoclonal antibodies. Samples of interfollicular epidermis, hair follicles and eccrine sweat glands were included as reference tissues. The CK reactivity in epidermoid cysts and milia is not restricted to CKs involved in epidermal-type differentiation, i.e. CK1, 5, 10 and 14, but in addition CK16, a hyperproliferative keratinocyte marker is suprabasally expressed. CK1 and 10 are other prominent suprabasal markers, while CK14 seems to be preferentially expressed in the basal cell layer. Of the non-epidermal CKs, only CK4 was focally or more extensively detected in about 50% of the cases. In terms of CK reactivity, keratinization of trichilemmal cysts corresponds to the keratinization of the anagen-phase hair follicle in the isthmus. The CK reactivity is again restricted to CK1, 5, 10, 14 and 16. However, the CK1 as well as CK10 reactivity is subject to serious limitations, since both CKs were only convincingly observed in foci of terminal differentiation. Eccrine hydrocystoma obligatorily expresses a complex CK set, including CK7, 8, 14, 18 and 19. This CK set perfectly corresponds to the CK composition observed in acini of eccrine sweat glands. In addition, a discontinuous CK4 and 16 reactivity was seen in about 50% of the sites, while CK1 and 10 displayed a strictly focal appearance. On the other hand, syringoma produces in its distinct structures, a CK pattern reminiscent of the one observed in eccrine sweat gland ducts and includes CK1, 5, 10, 14, 16 and 19. Finally, the CK expression pattern of pilomatricoma includes CK1, 8, 10, 14 and 19, and is reminiscent of the CK staining of hair bulb matrix cells differentiating in the keratogenous zone in the direction of hair cortex. The reactivity of CK1 and 10 was mainly restricted to foci of squamoid differentiation and also to transitional cells bordering on shadow cells, as far as it concerns CK10. Occasionally, CK7 and 16 were observed in individual cells or small cell groups. In our view these CK reactivity patterns are useful to judge the differentiation state reached in pathological conditions, but so far do not allow us unequivocally to determine the site of origin of these lesions.     

FINE NEEDLE ASPIRATION CYTOLOGY OF PILOMATRICOMA: CASE REPORT AND REVIEW OF LITERATURE

Pilomatricoma is a benign skin adnexal tumor that is underrecognized by fine needle aspirate, often times resulting in an overdiagnosis of malignancy. We present the fine needle aspirate of a 7-month-old child with a pilomatricoma of the cheek. The differential in this age group includes epidermal, dermoid, and branchial cleft cysts, thyroglossal duct remnants, granulomatous lymphadenitis, juvenile xanthogranuloma, and embryonal rhabdomyosarcoma. Recognition of the ghost cells and clinical correlation should allow accurate diagnosis of this entity.     

息肉性毛母质瘤1例

患者男,20岁。左侧背部出现息肉状损害半年余。皮肤科情况:左侧背部可见一约鸡蛋大半透明的大疱状损害,其中央可触及一约蚕豆大形状不规则结节,质坚,无明显压痛。手术切除物组织病理示:真皮内可见大量境界清楚的由影细胞、嗜碱性细胞和过渡细胞组成的细胞团块。诊断:息肉性毛母质瘤。     

Paratesticular pilomatricoma: a new location

The first case of pediatric paratesticular pilomatricoma is reported. Differential diagnosis with other more common lesions in such a site is the main issue. Conservative surgery is the treatment of choice. Follow-up is recommended, since most pilomatricomata are benign, but rarely malignant transformation may occur. Accepted: 16 February 2001     

The Immunohistochemical Patterns of the ��-Catenin Expression in Pilomatricoma

Background

Pilomatricoma is a benign follicular tumor that is composed of basaloid cells, transitional cells and shadow cells. β-Catenin is a 92-kDa protein, and it plays important roles in cell-cell adhesion at the cell membrane and signal transduction in the nucleus. β-Catenin has recently been shown to play an important role in the formation of hair follicle-related tumors, including pilomatricoma. However, the pattern and the intracellular localization of the β-Catenin expression are still controversial.

Objective

We wanted to evaluate the pattern and the intracellular localization of the β-Catenin expression in pilomatricoma by performing immunohistochemical staining.

Methods

Twenty-seven paraffin-embedded tissue samples that were diagnosed as pilomatricoma were immunohistochemically stained with β-Catenin antibody.

Results

Basaloid cells were found 15 samples of the total 27 pilomatricomas. All (15/15) of the basaloid cells strongly expressed β-Catenin, but the transitional cells and the shadow cells did not. In the basaloid cells, the nuclei and membranes showed prominent β-Catenin immunoreactivities, but the cytoplasm showed weak β-Catenin immunoreactivity.

Conclusion

This study confirmed that the nucleus and membrane of all the basaloid cells in the pilomatricomas showed a strong β-Catenin expression, but the transitional cells and shadow cells showed negative β-Catenin immunoreactivity.     

Optimal Timing of Surgical Excision in Pediatric Pilomatricoma: Association between Clinicopathological Features and Cosmetic Outcomes

BackgroundThe treatment of choice for pilomatricomas is surgical excision; however, data for the optimal timing of treatment and cosmetic outcomes are limited.ObjectiveThis study aimed to investigate the optimal timing of treatment in pilomatricomas by considering clinicopathological findings and cosmetic outcomes.MethodsSeventy-three pilomatricomas patients aged ≤15 years were retrospectively reviewed. Patients were classified into early excision (disease duration ≤12 months, group A) and delayed excision groups (disease duration >12 months, group B). Tumor characteristics, and histopathological features with evolutionary stages were assessed. Cosmetic outcomes were evaluated by the Modified Vancouver Scar Scale (MVSS), 5-point patient satisfaction score, and complication rates.ResultsGroup A showed better cosmetic outcomes than group B in the MVSS (1.53±1.22 vs. 3.68±1.84), 5-point patient satisfaction score (4.08±0.89 vs. 3.18±1.01), and complication rates (11.8% vs. 36.4%), respectively (p<0.05). Secondary anetoderma, tent sign, calcification, and late regressive stage (evolutionary stage IV) were more common in group B, (p<0.05). Moreover, evolutionary stages showed a positive correlation with mean MVSS (r=0.670, p<0.05).ConclusionEarly excision (disease duration ≤12 months) provides superior cosmetic outcomes compared to delayed procedures. Early recognition, diagnosis, and management for pediatric pilomatricomas is important to improve overall cosmetic outcomes.     

Pilomatricoma in childhood: a retrospective study from three European paediatric centres

Pilomatricoma is characterised as a common, slowly growing benign cutaneous tumour that appears generally within the first decades of life. The clinical diagnosis is frequently missed, especially by the paediatrician unfamiliar with these tumours. We present the experience gained in three European tertiary care paediatric centres with the treatment of pilomatricoma and also current data on the aetiology, clinical presentation and management. A retrospective study was carried out in 83 patients suspected for pilomatricoma during a 7-year period (1996–2002) at the departments of Paediatric Surgery of the Childrens University Hospital Federico II, Naples, Hospital San Bortolo, Vicenza and Aghia Sophia Childrens Hospital, Athens. The age range was from 10 months to 17 years, median age 8 years. All patients were treated by surgical excision and all specimens were examined by histopathological assessment. The follow-up varied from 5 months to 6 years. The correct diagnosis was made preoperatively in 68 patients (82%). The female/male ratio was 2:1. The sites of occurrence were the head (47.5%), especially in the periorbital region, the neck (9%), the upper limbs (35.5%), the inferior limbs (4%) and the thorax (4%). Each patient exhibited a single pilomatricoma except for two patients who had multiple lesions (2.4%). One of them had Steinert disease (myotonic dystrophy). No recurrences were observed during the follow-up period. Conclusion:Pilomatricoma is one of the most common cutaneous adnexal neoplasms in children. Surgical excision including clear margins and its overlying skin in most cases is the treatment of choice. The recurrence as well as malignant evolution is rare.