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Background: Cutaneous appendageal tumours belong to a diverse group of tumours with specific histo pathology.The aim of this study is to determine the pattern, age,gender and site distribution of Pilar differentiation tumours. Material & Methods: The study was conducted in the department of Pathology,Government Medical College Srinagar for a period of 18 months. It was an observational cross sectional study.Formalin fixed,paraffin embedded tissue sections were stained with hematoxylin and eosin stain for histopathological analysis. Results: A total of 112 cases of Pilar tumours were studied.108 were benign and 4 were malignant with male to female ratio of 1.07:1. The maximum number of benign cases were observed in 11 -20 years of age group and the malignant tumours age ranges from 35-45 years and the tumour usually presented in the eighth decade.Head and Neck was the most common site. Conclusion: Histopathological examination of Pilar Tumours is the gold standard to differentiate between benign and malignant tumours. It is also useful for exact categorization of cutaneous appendageal tumours.
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Objective:To analyze clinical and pathological features of childhood perforating pilomatricoma, and to explore its pathogenesis.Methods:A retrospective analysis was conducted on clinical and histopathological data from 29 children with perforating pilomatricoma in Department of Dermatology, Beijing Children′s Hospital from 2014 to 2020.Results:Among the 29 patients, 11 were males, and 18 were females, with a male-to-female ratio of 1∶1.64. Their age at onset ranged from 3 months to 14 years and 10 months, and the median age at onset was 4.58 years. The disease duration ranged from 2 months to 2 years, with an average of 8.72 months. The perforation occurred 2 days to 1 year and 6 months after the onset of pilomatricoma, with an average of 1.85 months. Ulceration occurred in 1 patient after the treatment with ichthammol, as well as in 3 patients after local scratching or bumping, and spontaneous ulceration without definite precipitating factors occurred in the remaining 25 patients. The average duration from the onset of disease to tumor perforation was 6.87 months. Skin lesions occurred on the face in 15 cases, on the lateral neck in 8, on the upper limb in 4, as well as on the scalp in 2. Perforating pilomatricoma clinically manifested as indurated subcutaneous nodules with crusts or ulcers, and was classified into 3 subtypes: ulcerative type (19 cases) , horny type (8 cases) , and crusted type (2 cases) . The tumor diameter ranged from 0.3 to 2.5 cm, with an average of 1.2 cm. Histopathological examination showed that the tumor was located in the superficial to middle dermis, and mainly consisted of basophils and ghost cells; in 19 cases, the tumor mass was extruded onto the skin surface through a perforated epidermal channel, and the epidermis around the perforation site was hyperplastic and invaginated into the dermis, forming epithelial tunnels surrounding the tumor; in 4 cases, the skin on the tumor surface was thinned and ruptured; in 6 cases, the perforation site could not be observed due to surgical separation of the epidermis and tumor. All lesions were resected, and no infection or recurrence was observed during the postoperative follow-up.Conclusions:Childhood perforating pilomatricoma mostly occurs on the face and neck, usually with rapid progress, and can be classified into ulcerative type, horny type and crusted type. Histological findings suggest that transepithelial elimination is an important mechanism underlying the occurrence of perforation in pilomatricoma.
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Abstract Pilomatricomas are benign tumors originating from the capillary matrix, which may present as solitary lesions or, less commonly, multiple. Myotonic dystrophy and familial adenomatous polyposis are the most frequently associated disorders with multiple pilomatricomas. There are few reports relating these tumors to other genetic syndromes. Rubinstein-Taybi syndrome is a rare autosomal dominant disorder characterized by intellectual disability and typical dysmorphic characteristics. There are five case reports relating to multiple pilomatricoma to Rubinstein-Taybi syndrome, an association that needs to be clarified. For this reason, we report the first case of multiple pilomatricoma in monozygotic twins with typical Rubinstein-Taybi syndrome.
Subject(s)
Humans , Rubinstein-Taybi Syndrome , Skin Neoplasms , Pilomatrixoma/blood , Hair DiseasesABSTRACT
Background: Skin adnexal tumors (SAT) encompass a wide spectrum of benign and malignant tumors that differentiate toward one or more adnexal structures found in normal skin. Overall incidence of SATs is low yet they can be challenging to diagnose. Our study aims at analysis of histopathological spectrum of various SATs according to age, sex and anatomic locations along with histopathological features.Methods: It was a retrospective cross-sectional study conducted over a period of 2 years from June 2017 to May 2019. All cases were retrieved from department registries and analyzed.Results: Out of the total 4013 biopsies, 25 cases were diagnosed as SATs comprising 44% of tumors with eccrine sweat gland differentiation followed by tumors showing hair follicle differentiation (32%), sebaceous differentiation (16%) and apocrine gland differentiation (8%). The age ranged from 4 years to 68 years and male: female ratio was 1:1.5. Most of the tumors were benign (84%) while only 16% were malignant. Pilomatricoma (19%) was the most common benign tumor while sebaceous carcinoma (75%) was the most common malignant tumor.Conclusions: Skin adnexal tumors are relatively rare neoplasm. Malignant cases are less common than benign ones. Histopathology is quite essential for diagnostic point of view.
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O pilomatrixoma constitui uma neoplasia benigna incomum de folículos pilosos, observado mais frequentemente em crianças. O tumor apresenta-se como um nódulo solitário e firme, recoberto com epiderme normal. É relatado o caso de uma paciente feminina de 26 anos com lesão na região temporal. Nos cortes histológicos, observou-se uma proliferação expansiva de ilhotas de células epiteliais configuradas de forma irregular, mostrando na área central sombra de núcleos perdidos (células- fantasma). O pilomatrixoma deve ser considerado no diagnóstico diferencial de nódulos cutâneos, especialmente aqueles de cabeça, pescoço e membros superiores.
Pilomatricoma is an uncommon benign neoplasm of hair follicles, most commonly seen in children. The tumor appears as a solitary and firm nodule, covered with normal epidermis. We report the case of a 26-year-old woman with a lesion in the temporal region. In histological sections, there was an expansive proliferation of islets of epithelial cells configured irregularly, showing the shadow of lost nuclei (ghost cells) in the central area. Pilomatricoma should be considered in the differential diagnosis of skin nodules, especially those of the head, neck, and upper limbs
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BACKGROUND: The treatment of choice for pilomatricomas is surgical excision; however, data for the optimal timing of treatment and cosmetic outcomes are limited.OBJECTIVE: This study aimed to investigate the optimal timing of treatment in pilomatricomas by considering clinicopathological findings and cosmetic outcomes.METHODS: Seventy-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.RESULTS: Group 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).CONCLUSION: Early 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.
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Humans , Anetoderma , Cicatrix , Diagnosis , Patient Satisfaction , Pilomatrixoma , Retrospective StudiesABSTRACT
Objective@#To summarize the clinical characteristics of pilomatricoma in children and analyze the preoperative diagnostic method.@*Methods@#468 cases (479 tumors) of pilomatricoma in children were treated in Department of Plastic and Maxillofacial Surgery, Wuhan Children’s Hospital from January 2013 to January 2018. All cases were confirmed by pathology after operation. There were 224 males and 244 females, aged from 3 months to 13 years and 10 months. The general situations, case histories, physical signs, auxiliary examinations and preoperative diagnoses of all patients were analyzed retrospectively. SPSS 22.0 software was used for statistical analysis of data. Median or mean±standard deviation was used for describing measurement data, and component ratio or rate was used for describing counting data.@*Results@#The peak age was 0-2 years old, accounting for 47.22%(221/468). The male to female ratio was 1∶1.09. The most common site of lesions was head(348 cases), followed by neck(76 cases), upper limbs(40 cases), trunk(10 cases) and lower limbs(5 cases). All patients had histories of subcutaneous mass, 85.90%(402/468) had histories of tumor growth, 16.24%(76/468)had pain, 7.91%(37/468) had histories of infection, 28.42%(133/468) had histories of previous treatment, and 12.18%(57/468) had histories of external stimulation. The masses were (1.2±0.6) cm(from 0.3 cm to 3.5 cm) in diameter. By inspection, 98.33%(471/479) of skin overlying the tumor were intact, and 63.29%(269/425) were pale blue. By palpation, 79.62%(375/471) of masses were hard, 100% adhered to the skin, 94.15%(451/479) didn’t cause tenderness, 31.73%(152/479) appeared "tent sign" , and 27.77%(133/479) appeared "teeter-totter sign" . The diagnostic accuracy rate of ultrasonographic examination was 75.24%(155/206). 31.55%(65/206) showed hypoechoic nodule containing internal hyperechoic dots, 27.67%(57/206) showed hyperechoic band with wide acoustic shadowing below. 88.83%(183/206) of Color Doppler Flow Imaging showed peripheral blood flow signal around the nodule, and the internal blood flow signal decreased or disappeared. The diagnostic accuracy rate of CT examination was 98.44%(126/128), 58.59%(75/128) of plain CT scans showed high density mass shadows of which the internal densities were homogeneous or heterogeneous. 39.84%(51/128) of plain CT scans showed soft tissue density mass shadows of which the internal densities were heterogeneous, and the dot high density shadows in different size scattered. The diagnostic accuracy rate of needle aspiration cytology was 61.54%(8/13). The preoperative diagnostic accuracy rate was 81.63%(391/479).@*Conclusions@#Typical pilomatricoma often occurs on a child’s head. The overlying skin is pale blue. The tumor is hard in texture, adheres to the skin, and appears a "tent sign" or "teeter-totter sign" . Ultrasonography and CT examinations suggest calcification in the subcutaneous lesion, which is helpful for preoperative diagnosis. Needle aspiration cytology can be used for differential diagnosis of atypical case.
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Malignant pilomatricoma (pilomatrical carcinoma) is a rare, locally occurring malignant tumor with a high rate of recurrence in the case of incomplete excision. This tumor has two characteristics. First, recurrences of pilomatrical carcinoma are common; second, distant metastasis is rare, but if it occurs, it is very fatal. It has characteristic features of high mitotic counts, cellular atypia, and local invasion. Although fine needle aspiration and excisional biopsy could help to confirm this tumor diagnosis, pathologic findings are critical. Pilomatricomas have some characteristic features in histological aspect, such as epithelial islands of basaloid cells and shadow cells or ghost cell. Also, various types of immunohistochemical staining are used to confirm the diagnosis. Despite the lack of clear surgical criteria, treatment is a wide local excision with histologically clear resection margins with or without adjuvant radiotherapy.
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Humans , Infant , Biopsy , Biopsy, Fine-Needle , Cheek , Diagnosis , Facial Neoplasms , Islands , Neoplasm Metastasis , Pilomatrixoma , Radiotherapy, Adjuvant , RecurrenceABSTRACT
Pilomatricoma or calcifying epithelioma of Malherbe is a benign tumor originating from the matrix of hair follicles. Clinically, these tumors are classified as familial, perforating, multinodular, exophytic, anetodermic, bullous, and giant pilomatricomas. The bullous variety is observed only in 3 to 6% of cases. Furthermore, multiple pilomatricomas are rare and are usually associated with genetic disorders. To date, only 13 cases of bullous pilomatricoma and 8 cases of multiple pilomatricomas have been reported in Korean dermatologic literature. Among these cases, no case of multiple bullous pilomatricomas has been reported. This report describes a 23-year-old healthy man who was diagnosed with multiple pilomatricomas with bullous features, based on clinical and histopathological findings.
Subject(s)
Humans , Young Adult , Hair Follicle , PilomatrixomaABSTRACT
El pilomatrixoma es una neoplasia anexial benigna, que habitualmente se presenta como pápula o nódulo subcutáneo, de crecimiento lento y asintomático. Es más frecuente en niños. El diagnóstico se basa en hallazgos clínicos, y la ecografía es útil como examen complementario. El tratamiento es su resección quirúrgica. Se presenta un caso infrecuente de pilomatrixoma quístico con verruga vulgar intraquística en un paciente adulto.
Pilomatricoma is a benign adnexal tumor, usually presenting as a slow growing asymptomatic subcutaneous papule or nodule. It is more common in children. The diagnosis is based on clinical findings, and ultrasound is useful as a complementary workup tool. Its treatment is surgical excision. We present a rare case of a cystic pilomatricoma with an intracystic verruca vulgaris in an adult patient.
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Humans , Male , Middle Aged , Skin Neoplasms/pathology , Warts/pathology , Pilomatrixoma/pathology , Hair Diseases/pathology , Skin Neoplasms/surgery , Skin Neoplasms/diagnosis , Pilomatrixoma/surgery , Pilomatrixoma/diagnosis , Hair Diseases/surgery , Hair Diseases/diagnosisABSTRACT
Objective To analyze the clinical and pathological features of childhood bullous pilomatricoma.Methods The clinical and pathological features were analyzed in 16 patients with bullous pilomatricoma,who visited Department of Dermatology of Beijing Children's Hospital from 2013 to 2017.Results Among the 16 patients,5 were males,and 11 were females.Their age of onset ranged from 4 months to 11 years,and the median age of onset was 8.5 years.Their course of disease ranged from 2 months to 4 years,and the average course of disease was 10 months.The tumors were found predominantly on the upper limbs (10 cases,including 7 on the upper arm,2 on the shoulder and 1 on the forearm),followed by the face (4 cases) and the neck (2 cases).These tumors manifested as limited pushable red lumps with blister-like appearance,and telangiectasia was observed on the surface of some lesions.The diameters of the lumps ranged from 0.5 cm to 3 cm,and hard nodules could be detected in the blisters by palpation.Under dermoscopy,uniform red background was observed in 16 cases,unstructured white area in 13,unstructured blue-grey area in 4,and liner or irregular branched vessels.Two or more dermoscopic features were observed in 15 patients.All the skin lesions were resected by surgery,and no recurrence was observed during the follow-up of 1-5 years.Histopathological examination showed that the tumors were located in the middle to deep dennis,and mainly consisted of basaloid cells and shadow cells,as well as transitional cells between the above two kinds of cells.Varying degrees of infiltration of inflammatory cells and hyperplasia and calcification of the fibrous connective tissue were observed in the tumor interstitium,with multinucleated giant cells in some areas.There were varying degrees of infiltration of inflammatory ceils,lymphangiectasis,reduction or absence of elastic fibers in the dennis between the epidermis and tumors.Conclusions Childhood bullous pilomatricoma mostly occurs on the upper limbs,face and neck.Histopathologically,the tumor consists of basaloid cells and shadow cells with lymphangiectasis and reduction of elastic fibers in the dermis.The main dermoscopic features are red background and unstructured white areas.Its prognosis is good after surgical excision.
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BACKGROUND: Although histological diagnosis of pilomatricoma is not difficult because of its unique histological features, cytological diagnosis through fine-needle aspiration cytology (FNAC) is often problematic due to misdiagnoses as malignancy. METHODS: We reviewed the cytological features of 14 cases of histologically-proven pilomatricoma from Korea Cancer Center Hospital, with a discussion on the diagnostic pitfalls of FNAC. RESULTS: Among 14 cases of pilomatricoma, 10 (71.4%) were correctly diagnosed through FNAC, and two (14.3%) were misdiagnosed as carcinoma. Cytologically, all cases had easily recognizable clusters of basaloid cells and foreign body-type multinucleated cells. Although ghost cells were also found in all cases, some were inconspicuous and hardly recognizable due to their small numbers. CONCLUSIONS: An accurate diagnosis of pilomatricoma in FNAC is feasible with consideration of clinical information and close examination of ghost cells.
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Biopsy, Fine-Needle , Diagnosis , Diagnostic Errors , Korea , PilomatrixomaABSTRACT
Objective To analyze the clinical and pathological features of chil dren's periorbital pilomatricoma.Methods The clinical and pathological data of 46 children patients who were diagnosed as periorbital pilomatricoma and underwent surgical treatment between February 2012 and February 2017 were analyzed.Results Of the 46 patients,18 males and 28 females (male:female =l.00:1.56),lesion location of superciliary arch was observed in 26 patients (56.5％),upper eyelid was in 18 patients(39.1％),and the lower eyelid in 2 patients(4.3％).Light microscope was used for pathological examination and the results showed that pilomatricoma was composed of two major cells,and they were basaloid cells and shadow cells.There were 36 patients presenting calcification,25 patients with inflammatory cell infiltration,and 24 patients with multinucleated giant cells.Furthermore,no recurrence or malignant transformation occurred in these patients after surgery.Conclusion Pilomatrixoma presents typical clinical and pathological feature,and it is not uncommon for children patients with periorbital pilomatricoma,so the clinical characteristics of the pilomatricoma and differential diagnosis of the periorbital tumors is important to improve the diagnostic accuracy.
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Objective To analyze the clinical and pathological features of chil dren's periorbital pilomatricoma.Methods The clinical and pathological data of 46 children patients who were diagnosed as periorbital pilomatricoma and underwent surgical treatment between February 2012 and February 2017 were analyzed.Results Of the 46 patients,18 males and 28 females (male:female =l.00:1.56),lesion location of superciliary arch was observed in 26 patients (56.5％),upper eyelid was in 18 patients(39.1％),and the lower eyelid in 2 patients(4.3％).Light microscope was used for pathological examination and the results showed that pilomatricoma was composed of two major cells,and they were basaloid cells and shadow cells.There were 36 patients presenting calcification,25 patients with inflammatory cell infiltration,and 24 patients with multinucleated giant cells.Furthermore,no recurrence or malignant transformation occurred in these patients after surgery.Conclusion Pilomatrixoma presents typical clinical and pathological feature,and it is not uncommon for children patients with periorbital pilomatricoma,so the clinical characteristics of the pilomatricoma and differential diagnosis of the periorbital tumors is important to improve the diagnostic accuracy.
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Pilomatricoma é uma neoplasia folicular benigna relativamente rara. Pode ocorrer em qualquer idade, mas menos de 13% dos casos acontecem em pessoas com mais de 50 anos, sendo mais comum em mulheres (1,5 a 2,5 mulheres:1 homem). A localização mais frequente é a região cervical, seguida das áreas frontais, periorbitárias e pré-auriculares (1). Clinicamente, apresenta-se como um tumor subcutâneo ou intradérmico, endurecido e de crescimento lento. O diagnóstico pré-cirúrgico é quase sempre difícil (3). O tratamento padrão é o cirúrgico, com margens de segurança amplas para minimizar o risco de recorrência nas variantes malignas (2,3). Relatamos um caso atípico e exuberante de múltiplos pilomatricomas em membros superiores em um paciente do sexo masculino de 55 anos (AU)
Pilomatricoma is a relatively rare benign follicular neoplasm. It can occur at any age, but less than 13% of cases occur in people over 50 years old, and it is more common in women (1.5 to 2.5 women: 1 man). The most frequent location is the cervical region, followed by the frontal, periorbital and pre-auricular areas (1). Clinically it presents as a hardened and slowly growing subcutaneous or intradermal tumor. Preoperative diagnosis is almost always difficult (3). The standard treatment is surgical, with wide safety margins to minimize the risk of recurrence in malignant variants (2,3). We report an atypical and exuberant case of multiple pilomatricomas in upper limbs in a 55-year-old male patient (AU)
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Humans , Male , Middle Aged , Pilomatrixoma/diagnosis , Pilomatrixoma/pathology , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Pilomatrixoma/surgery , Upper Extremity , Diagnosis, DifferentialABSTRACT
ABSTRACT Pilomatrixoma is a rare benign tumor, which usually affects young women in the head and neck region. The eyelid is a common site of the disease, though it is very rare in the tarsal conjunctiva.The pilomatrixoma has clinical pleomorphism, which confuses this disease with other similar conditions. The diagnosis is made by pathological examination in most cases. The treatment is surgical, performed by total excision of the lesion with clear margins and recurrence is very uncommon.We present an unusual case of probable recurrent pilomatrixoma in the left upper tarsal conjunctiva.
RESUMO O pilomatricoma é uma neoplasia benigna rara, geralmente acomete mulheres jovens, ocorrendo na região da cabeça e pescoço. A pálpebra é um local comum de aparecimento do pilomatricoma, contudo, seu aparecimento na conjuntiva tarsal é muito raro. O diagnóstico é feito pelo exame anátomo-patológico na maioria dos casos, pois seu pleomorfismo clínico o confunde com outras alterações. O tratamento é cirúrgico, realizado por meio da exérese total da lesão com margens livres. São raros os casos de recidiva. Apresentamos um caso atípico de provável recidiva de pilomatricoma, localizado na conjuntiva tarsal superior à esquerda.
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Humans , Female , Middle Aged , Pilomatrixoma/surgery , Pilomatrixoma/diagnosis , Pilomatrixoma/pathology , Conjunctival Neoplasms/surgery , Conjunctival Neoplasms/diagnosis , Conjunctival Neoplasms/pathology , Recurrence , Biopsy , Exotropia , Conjunctiva/surgery , Conjunctiva/pathologyABSTRACT
El niño con una masa cervical representa un desafío para el pediatra. Lo más frecuente en niños son los procesos benignos de origen infeccioso. Presentamos un niño con tumoración laterocervical posterior con diagnóstico de pilomatricoma, tumor benigno poco conocido en Pediatría . No presenta características específicas, por lo que su diagnóstico se base en el estudio de anatomía patológica. El único tratamiento posible es la exéresis completa. El pilomatricoma se debe incluir en el diagnóstico diferencial de adenitis cervical en niños para evitar intervenciones y tratamientos innecesarios.
Children with neck masses are an everyday challenge for pediatricians. Infectious diseases are the most common etiology in childhood. We report a child with a neck mass who was diagnosed with pilomatrixoma. It is a quite unknown benign skin tumor in pediatrics. It cannot be easily differentiated from other skin tumors, and biopsy is necessary to make the definitive diagnosis. Treatment of pilomatrixoma consists of total surgical excision. Pilomatrixoma should be considered in the differential diagnosis of cervical lymphadenitis in children to avoid unnecessary interventions and antibiotic treatment.