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Pilomatrixoma of the head and neck.

Pilomatrixoma is an uncommon neoplasm which occurs most frequently in the head and neck region. It usually presents as a slowly growing dermal or subcutaneous mass. Unfamiliarity with this lesion may lead the physician to mistake it for more common infectious or neoplastic processes. Most pilomatrixomas are benign and are adequately treated by local excision. Since a spectrum of clinical and histologic aggressiveness (pilomatrixoma carcinoma) has been noted in some lesions, a more aggressive surgical approach for such tumors is recommended. Two patients with pilomatrixoma are presented to emphasize the diagnostic confusion that can occur.

Aged↗

Pilomatrixoma of the head and neck in children.

Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign tumor of hair cell origin. It is an unusual, but not particularly rare, tumor. Fifty percent or more of pilomatrixomas occur in the head and neck, and 40% occur in patients under 10 years of age. Pilomatrixomas may be mistaken for cysts, calcified lymph nodes, calcified hematomas or hemangiomas, or parotid gland tumors. We describe our experience with 15 children, 1 1/2-12 years of age, in whom we have excised pilomatrixomas from the head and neck.

Child↗

Cytokeratin analysis of pilomatrixoma: changes in cytokeratin-type expression during differentiation.

The various structural components of pilomatrixoma (calcifying epithelioma of Malherbe) were studied for the expression of hair-specific (trichocytic) cytokeratins as well as epithelial cytokeratins, using immunoperoxidase and immunofluorescence microscopy of frozen sections as well as two-dimensional gel electrophoresis and immunoblotting. Trichocyte-type cytokeratins were detected in only a minor subpopulation of basophilic cells but more prominently in most "transitional" cells as well as in "shadow" cells. In contrast, antibodies against certain epithelial cytokeratins (including antibody KA1 against cytokeratins of stratified squamous epithelia and antibodies against cytokeratin 19) revealed an extensive but heterogeneous staining of basophilic cells. In regions of squamoid cells, epithelial cytokeratins in an unusual pattern were found. An antibody against vimentin was negative on pilomatrixoma cells. An antibody against desmoplakins decorated true desmosomes in basophilic and transitional cells. Biochemically, trichocytic cytokeratin polypeptides as well as epithelial cytokeratins 5, 6, 14, 16, 17, and 19 were positively detected. These results provide evidence in support of the notion of a trichocytic differentiation and probably derivation of pilomatrixoma. According to the cytokeratin expression patterns the majority of pilomatrixoma cells resemble the normal hair cortex lineage but some enter a pathway of squamous cell differentiation.

Adult↗

Fine needle aspiration diagnosis of pilomatrixoma. A monomorphic population of basaloid cells with squamous differentiation not to be mistaken for carcinoma.

In a case of pilomatrixoma, an initial fine needle aspiration (FNA) biopsy of a skin lesion yielded only a monomorphic population of mitotically active basaloid cells with squamous differentiation, without anucleated ghost cells, chronic inflammatory cells or foreign-body giant cells. These findings suggested a metastatic squamous-cell carcinoma or a malignant tumor of adnexal skin. The correct diagnosis was made by repeat FNA, which yielded the basaloid cells and ghost cells characteristics of pilomatrixoma; the FNA diagnosis was confirmed by subsequent excisional biopsy. This case demonstrates that pilomatrixoma should be considered in the differential diagnosis of skin aspirates that contain a dominant population of basaloid cells. The differentiation of pilomatrixoma from malignant lesions of the head and neck region is discussed.

Biopsy, Needle↗

Pilomatrixoma of the head and neck in children: a study of 38 cases and a review of the literature.

OBJECTIVES: To describe the clinical presentations and discuss the guidelines for surgical management of pilomatrixoma involving the head and neck in children. DESIGN: Retrospective study. SETTING: A tertiary care center. PATIENTS: Thirty-three patients, with a mean age of 4.5 years, underwent surgical treatment for pilomatrixoma (n = 38) between 1989 and 1997. INTERVENTION: All patients were treated surgically. In 34 cases, a direct approach was used to achieve complete removal of the lesion with (n = 11) or without (n = 23) skin resection. In the remaining 4 cases, an indirect approach via a parotidectomylike incision was used. RESULTS: In 88% of cases, the presenting symptom was a hard, slow-growing, subcutaneous tumor. The lesion was associated with pain and inflammation in 7 cases (18%) and abscess or ulceration in 4 cases (11%). Twenty-nine patients presented with single nodules and 4 presented with multiple occurrences. The lesions were located on the face (cheek, eyelid, or forehead) in 20 cases (53%), on the neck in 8 cases (21%), in the parotid region in 8 cases (21%), and on the scalp in 2 cases (5%). CONCLUSIONS: Pilomatrixoma is a rare, benign skin tumor, but practitioners should be aware of its clinical features. Diagnosis is usually easy based on clinical findings, but computed tomographic scan is helpful, especially in cases involving tumors located in the parotid region. Spontaneous regression is never observed. Complete surgical excision, including the overlying skin, is the treatment of choice.

Adolescent↗

Pilomatrixoma: a diagnostic pitfall in fine-needle aspiration biopsies. A review from a small county hospital.

A 10-year-old Hispanic girl presented with a 3-cm mass over her right parotid region. A fine-needle aspiration (FNA) was performed and the preliminary and final diagnoses were "suspicious for carcinoma, possible mucoepidermoid carcinoma." A subsequent computed tomography demonstrated an extra-parotid lesion attached to the skin. The FNA smears were reviewed and the diagnosis of a pilomatrixoma (PMT) was reached. The lesion was excised and the diagnosis of benign PMT confirmed. Pilomatrixomas are uncommon skin adnexal tumors most commonly found in the head and neck area of young adults and children. In 10 years there were 16 PMTs among 60,280 surgical pathology specimens in the pathology files of our 300-bed general county hospital. Pilomatrixoma is recognized as a diagnostic pitfall not only clinically but also on FNA cytology. On the physical examination, the bluish skin discoloration over the lesion so typical of PMT was overlooked. On FNA, the basaloid cells of PMT were mistaken for intermediate cells of mucoepidermoid carcinoma. The presence of anucleated squames was not properly recognized. These are usually absent in mucoepidermoid carcinoma and represent a hallmark of PMT. This case illustrates well the risk of misdiagnosing a pre-auricular PMT as a malignant tumor of the parotid gland.

Biopsy, Fine-Needle↗

[Insulated arm pilomatrixoma: A rare localization. A case report].

Pilomatrixoma also known as calcified epithelioma of Malherbe, is a rare benign cutaneous tumor. It is most commonly seen in the head and neck areas, during the first two decades of life. We report a 30-year-old female patient with a rare localisation of pilomatrixoma in the right arm. The pilomatrixoma was excised and at one year follow-up there has been no evidence of recurrence.

Adult↗

Multiple occurrence of pilomatrixoma.

Multiple occurrences of pilomatrixoma are rare. Although it has been known as a benign lesion, there are reports of some tumors that have histopathologic features of malignancy. A case of a 68-year-old Caucasian women with multiple pilomatrixoma is presented. She was operated on to remove three other pilomatrixomas on the arms 3 years after the initial excision from the face. Two years after excision of the lesions on the arms, no recurrences were evident.

Facial Neoplasms↗

Pilomatrixoma of the earlobe.

BACKGROUND: Pilomatrixoma most commonly occurs in the head and neck region, appearing primarily in the first two decades of life. The lesion is described as a tumor with differentiation toward hair cells and surgical excision is the treatment of choice. OBJECTIVE: We report an uncommon localization of a perforating type pilomatrixoma of 5 x 4 x 4 cm in size that developed from the earlobe. METHODS: Histopathological examination revealed pilomatrixoma. Total resection of the tumor was performed. The earlobe was reconstructed by forming a posterior-superior pedicled skin flap. RESULTS: No recurrence was observed and a good cosmetic result was obtained. CONCLUSION: Local recurrence does not generally occur if the tumor is completely removed. The case we present is of interest in having a history of trauma to the region and a relatively rapid growth with perforation of the overlying skin.

Child↗

Simultaneous multiple pilomatrixomas.

We report a 10-year-old girl with synchronous multiple pilomatrixomas. Pilomatrixomas are generally solitary tumors; synchronous multiple tumors are very rare. Multiple or recurring tumors may be found in association with various syndromes, and multiple tumors may be familial. Concurrent disorders were not identified in our patient and the etiology of her pilomatrixomas was not understood. Thorough examination of the patient and long-term follow-up are necessary with these tumors.

Child↗

[Pilomatrixoma of the head and neck in six cases].

Pilomatrixomas are benign neoplasms of the hair follicle, presenting as a mass in the head and neck region. Those arising from the parotid region may pose a diagnostic challenge. We present six patients (age range 6 to 68 years) with pilomatrixoma in the head and neck region. The lesions were located in the preauricular region in two cases, and in the periorbital region, the forehead, the neck, and in the dorsum of the nose in the other patients, respectively. The main complaint was a hard, slow-growing mass in all the cases. All the patients were treated surgically. Histopathologic diagnosis was pilomatrixoma. No recurrences or surgery-associated complications were observed in the follow-up period.

Adolescent↗

Pilomatrixoma: a common, benign childhood skin tumour.

OBJECTIVE: To review recent experience with pilomatrixoma, particularly with respect to diagnosis. DESIGN: Chart review for an 8-year period. SETTING: A university-affiliated children's hospital. PATIENTS: Fifty children with 51 histologically proven pilomatrixomas. INTERVENTION: Excision of the lesion. MAIN OUTCOME MEASURES: Clinical presentation, diagnosis and pathological findings. RESULTS: The clinical presentation was typically that of an asymptomatic superficial mass that increased slowly in size and was located in the head and neck (78%) or chest and upper limbs. In one case the mass increased rapidly over 2 weeks and changed in colour from red to purple with areas of pink and tan. The referring diagnosis was incorrect in 94% of cases, and the preoperative diagnosis was incorrect in 57%. Pathologically the tumours were situated in the dermis or subcutaneous tissue. None were cystic, but several had areas of calcification. None of the tumours recurred after excision, but two patients had multiple lesions at different locations and times. CONCLUSIONS: Pilomatrixomas in children are simple to treat successfully but have variable clinical presentation. Early diagnosis is important so that unnecessary, aggressive surgery can be avoided.

Child↗

Pigmented malignant pilomatrixoma: report of a case and review of the literature.

An unusual variant of malignant pilomatrixoma displaying melanization of epithelial elements is described. Melanization is a rare event even in the benign form of this adnexal neoplasm. Previously reported cases of malignant pilomatrixoma are reviewed; none containing pigment have been previously reported to our knowledge. Possible etiologies for lack of pigment in most benign and malignant pilomatrixoma are discussed.

Aged↗

Fine needle aspiration cytologic findings in four cases of pilomatrixoma (calcifying epithelioma of Malherbe).

Four cases of pilomatrixoma studied by fine needle aspiration (FNA) biopsy prior to resection and histologic diagnosis were reviewed to identify the cytologic features useful in making the FNA cytodiagnosis. Cytopathology had initially correctly diagnosed two cases while misdiagnosing one as a carcinoma and one as a suspected carcinoma. The aspirates from all cases contained either clustered or isolated basaloid cells, with variably sized nuclei and prominent nucleoli. "Ghost cells" were also present in most smears stained with the Giemsa stain and could thus be very helpful for making the FNA diagnosis of pilomatrixoma. The occurrence of either foreign-body giant cells or calcium deposits in the aspirates could also contribute to suggesting the correct diagnosis of pilomatrixoma in some cases.

Adult↗

Pilomatrixoma as a diagnostic problem: the Edinburgh experience.

The purpose of this retrospective study was to review the paediatric experience in Edinburgh of a relatively benign skin tumour. The study group consisted of children less than 13 years of age with a pathological diagnosis of pilomatrixoma over the period between January 1980 and December 1989. The results reaffirmed a female preponderance. The majority of lesions had been present for less than 1 year at the time of removal but were mainly misdiagnosed at the time of primary consultation or at removal. Although pilomatrixoma has a 'typical' appearance pathologically there are problems with preoperative diagnosis. Sites of occurrence are comparable with the dermoid type of cyst and the possibility must be considered of an embryological origin for pilomatrixoma.

Child↗

Pilomatrixoma: a pitfall in the aspiration cytology of skin tumors.

The aspirates from six tumors of pilomatrixoma were cytologically misinterpreted. The fine needle aspiration findings of these six tumors are described. The common cytologic features of pilomatrixoma are clusters of tightly arranged cells and numerous single naked nuclei. The nuclei of cells in clusters as well as the naked nuclei are vesicular, with evenly distributed chromatin and distinct nucleoli. In addition, "ghost" cells and multinucleated giant cells present in some smears can support the cytologic diagnosis of pilomatrixoma.

Biopsy, Needle↗

Pilomatrixoma--accuracy of clinical diagnosis.

BACKGROUND/PURPOSE: Pilomatrixoma is a common tumor of skin appendages in children. The aim of the study was to assess the accuracy of clinical diagnosis and factors contributing to misdiagnosis. METHODS: A retrospective case note review of patients who had pilomatrixoma excised during a 5-year period in a tertiary referral children's hospital in the UK. RESULTS: From 75 patients, 78 pilomatrixomata were excised. The diagnosis was achieved preoperatively in 46% of patients. Other diagnoses included sebaceous and dermoid cysts, foreign body reaction, calcification in lymph gland, and fat necrosis. CONCLUSION: Factors contributing to misdiagnosis include cystic lesions with varying consistency, punctum-like appearance, atypical location, and absence of clinically recognizable calcification. Despite close excision, the recurrence rate is low.

Adolescent↗

Fine needle aspiration (FNA) cytology of pilomatrixoma: report of 14 cases and review of the literature.

We describe 14 cases of pilomatrixoma which were diagnosed preoperatively on FNA cytology. In contrast to the literature, our series showed male preponderance and some of the tumours in unusual locations such as thigh and breast. In three patients the tumours were larger than 5 cm. The clinical diagnosis varied from tumour not otherwise defined to sarcoma. The cytologic presentation had features which allowed a correct diagnosis in all cases and included basaloid cells surrounded by delicate pink fibres, shadow cells, giant cells, naked nuclei and calcium deposits. It is concluded that the FNA cytology of pilomatrixoma is characteristic and will allow a conclusive diagnosis even in cases with an aberrant clinical presentation.

Adolescent↗