Massive Pilomatrixoma of the Scalp: A Case Report.

Pilomatrixoma, also called epithelioma of Malherbe, is a benign neoplasm derived from hair follicle matrix cells. It usually presents as a solitary mass in the head and neck region and is more frequent in children and young adults, females, and the Caucasian population. Lesions equal to or greater than 5 cm are categorized as giant pilomatrixomas. We present a case of a 75-year-old female, with no known medical history, who was brought to the emergency department (ED) after falling on the street. She had a giant soft head tissue tumor, severe anemia due to intralesional chronic small hemorrhages and folates and cobalamin deficiencies, and delirant speech. The anatomopathological result of the biopsy of the tumor revealed to be a pilomatrixoma. The patient was then referred to plastic surgery, with complete excision of the tumor. After surgery, she was transferred to the psychiatric team, who assumed the delirant speech to be in the context of schizophrenia. She was discharged four months after admission.

Post-traumatic pilomatricoma presenting as rapidly enlarging pediatric scalp mass.

Pilomatricomas (PM) are benign neoplasms that arise from hair follicle matrix cells. They are one of the most frequently excised pediatric skin masses. A typical clinical presentation is a slow growing, painless, firm, superficial mass in the head and neck of a child. We present a rare presentation of PM. A preschool aged girl presented with an enlarging scalp mass following a history of minor blunt trauma to the area. Two months prior, the child sustained a ground level fall and subsequently developed a painless "bump" at the site of injury on the scalp. The ``bump" was initially the size of a "mosquito bite" and enlarged to the size of a "ping pong ball". Given the rapid progression and history of trauma, there was a broad differential diagnosis. An extensive workup including sonography, computed tomography, MRI, and biopsy were performed. The final pathologic diagnosis was confirmed as pilomatricoma. An atypical presentation of PM in a child can mimic a wide variety of pathology. Our case demonstrates unusual features of rapid enlargement and preceding trauma. With the widespread use of sonography for evaluation of superficial lesions, the radiologist may be the first to suspect the diagnosis of PM. Knowledge of the typical imaging findings in PM can be valuable, especially in more unusual cases.

Clinicopathological and immunohistochemical characteristics of bullous pilomatricoma: A retrospective, single-center study, and comparison with ordinary pilomatricoma.

BACKGROUND: Bullous pilomatricoma is a rare variant of pilomatricoma. As it has been published in sporadic case reports, a limited understanding of its clinicopathological characteristics restricts its effective diagnosis and treatment. OBJECTIVES: This study aimed to analyze the clinicopathological and immunohistochemical characteristics of bullous pilomatricoma to better understand the bullous transformation of pilomatricoma. METHODS: The authors conducted a retrospective study of 12 patients with bullous pilomatricoma and compared their clinical, histopathological, and immunohistochemical data with those of patients with ordinary pilomatricoma. RESULTS: Bullous pilomatricoma showed no sex preference, with a mean onset age of 31.2 years. The common sites were the upper extremities and trunk. Bullous pilomatricoma had a shorter disease duration, a larger diameter, and a greater tendency to increase in size than those of ordinary pilomatricoma. Histopathologically, bullous pilomatricoma had a shorter duration, lesser calcification, more mitotic figures, and distinct dermal features from those of ordinary pilomatricoma. Immunohistochemically, the expression of Matrix Metalloprotease (MMP)-2, MMP-9, vascular endothelial growth factor receptor-3 (VEGFR-3), and VEGF-C was elevated. STUDY LIMITATIONS: The study was retrospective, and the sample size was small. CONCLUSION: The distinctive features of bullous pilomatricoma potentially result from dermal changes associated with the release of angiogenic factors and proteolytic enzymes. This comprehensive analysis provides novel insights into the clinical features and pathogenesis of bullous pilomatricoma.

Unusual presentation of pilomatricoma: A case report and comprehensive review of the literature.

Pilomatricoma is a benign proliferative lesion of skin appendages that often affects the head, upper limbs, and lower limbs. The clinical appearance of the lesions is that of asymptomatic nodules measuring less than 3 cm. pathologically, these skin lesions show the presence of basaloid cell islands, eosinophilic cytoplasmic cells without nuclei, as well as hemorrhage and calcification. In this study, we present the case of an 8-year-old girl with a 5 × 5 cm skin lesion on the forearm, which lacked the typical firmness associated with pilomatricoma lesions during examination. After biopsy, the lesion was confirmed to be pilomatricoma. Furthermore, we have reviewed studies documenting pilomatricoma lesions with atypical clinical features. Based on reports of different clinical manifestations of pilomatricoma in these studies, we suggest that the clinical diagnosis of pilomatricoma should not be limited to the typical presentation of these lesions. In cases where the lesions exceed 3 cm in size, display cystic characteristics, are painful, or resemble keloids, consideration should also be given to the possibility of pilomatricoma.

Giant pilomatrix carcinoma of the scalp with intracranial extension: A case report.

Key Clinical Message: A very rare skin cancer. Malignant variant of pilomatricoma. It is unique because of its highly aggressive behavior. Responds poorly to chemotherapy and radiotherapy. Treated with a wide surgical excision with safe margins. Abstract: Pilomatrix carcinoma is uncommon and locally aggressive tumor of the hair follicle matrix. It is a malignant variant of pilomatricoma. If left untreated for a long time may attain massive size and has the potential for distant spread. Only few cases with brain extension have been reported in the literature. Diagnosis of these tumors is established by histopathology. Although pilomatrixoma and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. The tumors respond poorly to chemotherapy and radiotherapy. Thus, recommended treatment is a wide surgical excision with safe margins. Herein, we report the case of a 39-year-old African woman with pilomatrix carcinoma of the scalp eroding the skull bone with intracranial extension.

Pilomatrixoma of the Arm: A Case Report and Review of Literature.

Pilomatrixoma (PMX), also known as calcifying epithelioma of Malherbe, is a rare benign neoplasm that arises from the hair matrix cells, commonly in the head, neck, and upper trunk regions, infrequently affecting upper and lower extremities. It has to two peaks of presentation: under 20 years of age or between 50 and 65 years of age, slightly more common in females. The neoplasm exhibits diverse clinical manifestations and is frequently subject to misdiagnosis with alternative dermatological diseases. We present an atypical case of PMX affecting the upper extremity of a 62-year-old female patient. Surgical removal of the affected tissue under local anesthesia was performed, and subsequent histopathological analysis confirmed the presence of PMX. Based on the literature search we performed, we found out that this pathology is underreported in Jordan, with only one study published describing this tumor in the maxillofacial region. Physicians should be aware of this condition and its different presentations to include it in the differential diagnosis of suspected cases to provide the appropriate management and follow-up.

A systematic review of Pilomatrix carcinoma arising from the previous scar site of Pilomatrixoma.

Pilomatrix carcinoma is a rare tumor arising from the hair follicle matrix cells most commonly seen in the head and neck region. Also known as "calcified epithelial carcinoma of Melherbe," it was first reported in 1980 by Lopansri and Mihm. Since then till date to the best of our knowledge only around 125 cases were reported in literature, of which only 11 cases were reported to arise from histologically proven areas of previous pilomatrixoma which is the benign variant. One such case is being reported here along with the review of literature. A 50-year-old man presented with a swelling in the nape of his neck since 6 months, which was gradually increasing in size. He had a history of similar swelling at the same site 18 months back for which he underwent a surgery at a center outside. Final histopathology report was suggestive of pilomatrixoma with negative margin. Wide local excision of the tumor with 3 cm margin, placement of surgical clips followed by a primary closure was done. The final histopathology report is suggestive of pilomatrix carcinoma. The patient has no recurrence in 6 months follow-up. The differential diagnosis of pilomatrix carcinoma should be considered in cases of recurrent skin tumors. Wide local excision is the preferred treatment. Re-excision should be done in margin positive cases and cases where simple excision was done due to improper preoperative diagnosis. Due to the rarity of the disease, adjuvant treatment is not properly defined.

A Large Mass Displaying a Malignant Character on the Forearm: A Rare Case of Upper Extremity Pilomatrixoma.

In this study, a 50-year-old male patient had a painless swelling on his right forearm. The lump on the forearm started one year ago and increased in size in the last two months. The mass was 3x6 cm and had a malignant appearance on radiological imaging. The case was reported as pilomatrixoma in the histopathological examination after marginal excision. In this case report, we emphasized that pilomatrixoma is one of the diagnoses we considered in mass formations that can be seen in the upper extremity, although rare. The large mass displaying a malignant character in radiological imaging can be pilomatrixoma, and the Tru-cut biopsy before the final surgery may help diagnosis by preventing the surgeons from aggressive surgical treatment. The marginal excision shall be enough in the definitive treatment. With this study, we aimed to discuss the place of pilomatrixoma in the orthopedic literature, which is published chiefly by otolaryngology, pathology, and dermatology clinics and lacks in the orthopedic literature because it rarely involves the extremities.

Multiple giant pilomatricomas of the scrotal: A rare case report from Syria.

Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a cutaneous tumor originating from the hair matrix that commonly affects children. Pilomatrixoma is usually solitary and most commonly found on the face, neck, and upper torso, while the scrotum is considered a very rare site. We report a rare location and manifestation of pilomatrixoma as multiple, large, firm, calcified scrotal masses in a 32-years old man. An excisional biopsy was performed, and the diagnosis was confirmed.

Pilomatrixoma of the Male Breast: Case Report and Literature Review.

Pilomatrixoma is a rare benign skin adnexal tumor arising from the hair follicles. Its occurrence in the male breast is extremely uncommon with very few cases reported worldwide. Pilomatrixoma may masquerade as a malignant tumor due to the presence of foci of calcification. We present a case of a 51-year-old man with a six-month history of a firm-to-hard mass in his right breast. The mammographic and sonographic features were suggestive of skin-related lesions. The lesion was excised. Histopathology confirmed the diagnosis of pilomatrixoma.

Rare Cutaneous Manifestations of Erdheim Chester Disease: A Case Report and Literature Review.

Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed. We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD.

Childhood pilomatrixoma mimicking malignant small round blue cell tumor with positivity for CD99: Potential pitfall in cytology.

Pilomatrixoma is a relatively rare benign skin appendageal tumor, often presenting in the pediatric age group as a nodular lesion and most commonly involving the head and neck, making it amenable to primary fine needle aspiration (FNA) diagnosis. We report the clinical and histopathological findings of two cases of pilomatrixoma in children, both of which were initially misdiagnosed as small round blue cell tumors due to high cellularity and misinterpretation of the proliferating basaloid cells. Histopathology revealed basal cell proliferation and mitoses indicating that they were progressive, early lesions. The first case showed membranous positivity for CD99 which prompted a diagnosis of Ewing sarcoma. Awareness of the morphological spectrum including positivity for CD99 and careful evaluation of cell block histology could have averted the misdiagnosis. Pilomatrixoma should be included as an important differential diagnosis when faced with primitive-appearing cells on FNA, especially in children with mass lesions in the head and neck region.

Hand Pilomatrixoma: A Rare Localization.

Pilomatrixoma, also known as calcified epithelioma of Malherbe, is the most common tumor of the hair follicle. It is a benign tumor in a young adult. Pilomatrixoma is most commonly located in the head and neck. It is usually misdiagnosed and confused with other skin lesions. The authors report a rare localization of this tumor in the left hand in a 40-year-old patient treated surgically without recurrence after four years of follow-up.

Cytological Diagnosis of Pilomatrixoma and its Diagnostic Pitfalls.

Background: Pilomatrixoma (PMX) is a relatively uncommon benign cutaneous neoplasm arising from skin adnexa. It presents as a subcutaneous asymptomatic nodule mostly in the head and neck region and is frequently misdiagnosed by the clinicians. Although easily diagnosed on histopathology, the cytologic features of PMX are less distinctive, depending on the stage and evolution of disease and may mimic other benign or even malignant lesions. Aim: To study the cyto-morphological features of this uncommon neoplasm and identify its potential diagnostic pitfalls on fine needle aspiration cytology (FNAC). Material and Methods: Archival records of histopathologically diagnosed Pilomatrixoma were analyzed during study period of 2.5 years. Clinical diagnosis, preoperative FNA characteristics, and histopathological details were studied in each case. Cytologic pitfalls resulting in misdiagnosis of PMX cases on FNAC were evaluated in discordant cases. Results: The series showed male preponderance, with head and neck being the commonest site. Out of 21 histopathologically proven cases of PMX, cytological correlation was available in 18 cases. A correct cytologic diagnosis of PMX/adnexal tumor was rendered in 13 cases. Erroneous diagnosis was given in 5 cases mainly because of the predominance of one component over the other or non-representative-aspirated material. Conclusion: The present study highlights the importance of careful screening of FNAC smears keeping in mind the variability in the relevant cytologic features of PMX and creates awareness about the lesions that can mimic Pilomatrixoma resulting in diagnostic dilemma.

A Rapidly Growing Forearm Pilomatricoma in an Elderly Patient.

Pilomatricoma is a benign skin tumor of epithelial hair matrix cells that typically presents as a solitary nodule on the head or upper trunk. It occurs most often in children and young adults. While considered uncommon in middle-aged and elderly patients, there are reports of elderly patients with histopathologically diagnosed pilomatricomas; however, these cases primarily occurred on the face. We present a case of an 88-year-old female with a history of non-melanoma skin cancer who presented with a new, rapidly enlarging, biopsy-proven pilomatricoma on the forearm. This case highlights a unique age of onset and location for this skin tumor, suggesting that pilomatricomas are not limited to children and young adults and should be considered in the differential diagnosis of rapidly growing skin lesions in elderly patients. Diagnosis should be confirmed with biopsy in elderly patients, as pilomatricomas may mimic malignant skin lesions.

Early-Stage Pilomatricoma with Cystic Features: A Case Report and Literature Review.

A conventional pilomatricoma presents as an asymptomatic, firm, subcutaneous nodule, commonly in the head, neck, and extremities of the paediatric population. However, its variants show diverse clinical features, which often pose a diagnostic challenge to clinicians. We recently treated an unusual case of pilomatricoma with cystic features in the upper chest of a 9-year-old girl, which manifested as a single solid tumour floating in a serous-filled sac formed by thick fibrous tissue. Herein, we present novel clinicopathological features of an early-stage pilomatricoma that should be clinically differentiated from a cutaneous abscess.

A rapidly growing pedunculated pilomatrixoma of the eyelid.

A pilomatrixoma is a benign tumor originating from a hair follicle that most frequently occurs in the head-and-neck region. It usually presents as a subcutaneous, slow-growing, nodular, painless firm mass. There are few reported cases on eyelid pilomatrixoma. We reporting an unusual presentation of a rapidly growing pedunculated eyelid pilomatrixoma in a 29-year-old female patient. Surgical excision was performed, and histological examination showed a cavity containing proliferating cords of basaloid cells differentiated into eosinophilic keratinized shadow cells confirming the pilomatrixoma diagnosis. Only a few cases with pedunculated eyelid masses have been reported in the literature; pedunculated lesions can be misdiagnosed as vascular tumors or malignancies. Therefore, pilomatrixoma should be considered in the differential diagnosis of such a presentation. A complete excisional biopsy of the mass is diagnostic and therapeutic.

Periocular pilomatrixoma in childhood: Clinical feature and differential diagnosis.

PURPOSE: To explore the clinical characteristics, imaging features, and differential diagnosis of periocular pilomatrixoma in children and provide evidence for clinical diagnosis and treatment. METHODS: Retrospective analysis of the clinical characteristics, preliminary diagnosis, imaging features, treatment, and follow-up of the cases of pediatric periocular pilomatrixoma treated at our hospital. RESULTS: A total of 59 patients from 4 months to 13 years of age (median age 4 years) were collected; 18 cases (30.51%) were misdiagnosed as other diseases in preliminary diagnoses. Seven cases underwent computed tomography (CT) examination, with CT value ranging from 63.4 Hounsfield Units (HU) to 952.0 HU (median value 151.0 HU). Six cases underwent magnetic resonance imaging (MRI) examination; two patients underwent an enhanced scan. The results showed that the rim of the lesion was enhanced, but the contents were not enhanced. All patients underwent surgical treatment. No recurrence was found from 1 month to 5 years of follow-up. CONCLUSIONS: Periocular pilomatrixoma is a relatively common tumor in children, which can easily be misdiagnosed clinically as other diseases, such as sebaceous and dermoid cysts. Although not generally recommended, CT can be of significant value in the diagnosis of pilomatrixoma. MRI is of little value in the diagnosis of this disease. If CT images show high or density, the possibility of pilomatrixoma should be considered.