Report of Basal Cell Carcinoma Occurring With a Desmoplastic Trichilemmoma Successfully Treated With Mohs Micrographic Surgery.

Desmoplastic trichilemmoma, an uncommon variant of trichilemmoma, is a benign adnexal neoplasm originating from the outer root sheath of a hair follicle, which has rarely been associated with atypical basaloid proliferations, including basal cell carcinoma. In this patient case, a 67-year-old female presented to our dermatology clinic for a skin check. On physical examination, a pearly, pink papule was noted on the vertex scalp, and a biopsy was obtained to rule out malignancy. Histologic examination of the lesion favored a desmoplastic trichilemmoma; however, a basaloid neoplasm could not be ruled out. Subsequently, the patient underwent Mohs micrographic surgery, and upon examination of the Stage I Mohs slides, superficial basal cell carcinoma was identified within the lesion. This case serves to further strengthen the known association between basal cell carcinoma and desmoplastic trichilemmoma. In addition, it demonstrates that the presence of basal cell carcinoma may not be observed on the initial biopsy of these lesions, underscoring the utility of complete surgical excision.

Utilizing PTEN immunohistochemistry as a screening test for Cowden syndrome.

OBJECTIVES: Cowden syndrome (CS) is a multisystem disease with an elevated lifetime risk of internal malignancy. We aim to assess the role of PTEN immunostain as a screening test for CS in a variety of common CS-associated neoplasms, with a particular focus on cutaneous tumors. METHODS: We retrospectively searched for patients meeting criteria for CS and/or demonstrating germline PTEN mutation from 2008 to 2022. We then performed PTEN immunostains on tumors of these patients as well as control cases. RESULTS: Our study included 30 patients with CS who had a total of 25 CS-associated malignancies (13 thyroid, 8 breast, and 4 endometrial carcinomas). Specifically, there were 11 patients with biopsy-confirmed CS-associated cutaneous neoplasms, including 1 patient with multiple trichilemmomas and 3 with multiple sclerotic fibromas. In total, 45 CS-associated tumors (6 trichilemmomas, 7 sclerotic fibromas, 5 thyroid carcinomas, 18 adenomatous thyroid nodules, 6 breast carcinomas, and 3 endometrial carcinomas) and 31 non-CS cases (9 trichilemmomas, 5 sclerotic fibromas, 8 adenomatous thyroid nodules, and 3 thyroid, 3 breast, and 3 endometrial carcinomas) were available for PTEN immunohistochemical staining. PTEN expression was lost in 43 (96%) of 45 CS-associated lesions and retained in 30 (97%) of 31 sporadic tumors. The overall sensitivity and specificity of PTEN loss of expression as a screening test for CS were 96% and 97%, respectively. CONCLUSIONS: PTEN immunohistochemistry on CS-associated tumors, especially trichilemmomas, can serve as a readily accessible and cost-effective screening test for CS.

Pigmented Desmoplastic Trichilemmoma Arising in Nevus Sebaceous: A Potential Mimicker of Pigmented Basal Cell Carcinoma.

Introduction: Trichilemmomas are a form of benign adnexal neoplasm with differentiation toward the follicular outer sheath. Nevus sebaceous (NS) of Jadassohn is a congenital malformation characterized as a nonhereditary hamartoma of the adnexal structures of the skin, mainly the pilosebaceous unit. NS represents a fertile field for the development of secondary adnexal neoplasms, commonly benign but occasionally malignant. To our knowledge, one case of a pigmented desmoplastic trichilemmoma (DT) has been reported in the English literature. Case Presentation: We report a case of a 36-year-old male with pigmented DT that developed on long-standing congenital NS, mimicking pigmented basal cell carcinoma (BCC), which was successfully excised with 4-mm margins. Conclusion: Pigmented DT may mimic pigmented BCC on clinical and dermatoscopic examination; therefore, histological evaluation is always recommended to confirm the diagnosis.

Trichilemmoma Arising in a Sebaceous Nevus Successfully Treated with Cryotherapy.

Nevus sebaceous of Jadassohn (NSJ) is a benign congenital hamartoma composed of follicular, adnexal appendages and epidermal hyperplasia. Various secondary neoplasms, mostly adnexal in origin, can arise within nevus sebaceous. We report the case of a 16-year-old female who had NSJ associated with trichilemmoma, which was successfully treated with cryotherapy. We found cryotherapy to be an effective, satisfactory therapy, with acceptable cosmetic results, especially for patients who have visible lesions that can be easily followed.

Crown Vessels of Desmoplastic Trichilemmoma: Use of Dermoscopy with Pathological Correlation.

Desmoplastic trichilemmoma is a rare histological variant of a benign tumor of the pilosebaceous hair follicle that often clinically appears as similar in appearance to other cutaneous lesions. Here, an 81-year-old male patient with desmoplastic trichilemmoma found on the left zygoma is presented. During the dermatoscopic evaluation of the neoplasm, crown vessels with radial distribution in the periphery were displayed. Histopathologic evaluation revealed peripheral palisading lobules of tumor cells surrounded by sclerotic hyalinized stroma displacing the vessels of the tumor. This case highlights the value of using dermoscopy for improving the clinical diagnosis of desmoplastic trichilemmoma. These findings highlight a need to further investigate the diagnosis of desmoplastic trichilemmoma when crown vessels are displayed during the clinical evaluation.

Clear Cell Acanthoma: A Review of Clinical and Histologic Variants.

Degos and Civatte first described clear cell acanthoma (CCA) in 1962 and later in a review article found that, in most instances, the lesion was a solitary red-brown dome-shaped papule that involved the distal lower extremity. The first morphologic variant of CCA was reported as a "giant form of the acanthoma of Degos" which measured 45 × 40 mm, about twice the size of the largest CCA documented earlier. Since then, many variants of CCA have been described, including polypoid, pigmented and atypical. Herein, we describe a new variant of CCA and add another example of the polypoid variant to the literature. The new variant exhibits cellular features of trichilemmoma but architecturally differs from it. We also attempt to broaden the list of CCA variants summarized by Tempark and Shwayder by adding ours and a few more examples of CCA. The new variants of CCA include verrucous, linear, subungual and trichilemmal.

Desmoplastic trichilemmoma arising in a nevus sebaceous: a case report.

Nevus sebaceous is a congenital hamartoma of cutaneous structures, commonly associated with the development of secondary neoplasms. Desmoplastic trichilemmoma is characterized by the typical features of trichilemmoma (proliferation of columnar clear cells surrounded by cells forming a palisade resting on a thickened eosinophilic basement membrane) but contains a fibrous stroma with bands of epithelial cells, generally in the central zone. To the best of our knowledge, only 5 cases of desmoplastic trichilemmoma arising in a nevus sebaceous have been reported previously. Herein we report an unusual case of desmoplastic trichilemmoma arising in a nevus sebaceous and review the literature.

A Case of Eyelid Desmoplastic Trichilemmoma

PURPOSE: Desmoplastic trichilemmoma is a rare variant of trichilemmoma first described in 1990. Desmoplastic trichilemmoma on the eyelid has not been previously reported in Korea. We report our experience with a case of desmoplastic trichilemmoma of the eyelid. CASE SUMMARY: A 72-year-old male patient presented with a round mass on the upper eyelid, which was noticed 1 year prior and caused irritation and itching. Excisional biopsy of the mass was performed. Histopathological examination revealed findings suggestive of a desmoplastic trichilemmoma. There was no local recurrence during the 2-year follow-up. CONCLUSIONS: Desmoplastic trichilemmoma should be considered when determining the type of eyelid mass.

Simultaneous Development of Three Different Neoplasms of Trichilemmoma, Desmoplastic Trichilemmoma and Basal Cell Carcinoma Arising from Nevus Sebaceus.

Nevus sebaceus is a hamartoma of the sebaceous gland that occurs congenitally, from which various secondary tumors can arise with a prevalence of 5%-6%. Benign neoplasms commonly arise from nevus sebaceous, but they have a very low malignant potential. Two neoplasms may occasionally arise within the same lesion, but it is rare for three or more neoplasms to occur in a nevus sebaceus simultaneously. A 61-year-old male patient was admitted to our hospital for a 4 cm×2.5 cm growing tumor in a verrucous form arising within a periauricular nevus sebaceus in the post auricle of the left ear that had developed 30 years earlier. The nodule was diagnosed as 3 different types of tumors: trichilemmoma, desmoplastic trichilemmoma, and basal cell carcinoma. To our knowledge, this is the first report of the coexistence of three different tumors arising from nevus sebaceous. It contain malignant neoplasm also. Surgeons should be aware of the need for close monitoring and early complete surgical excision of sebaceous nevus in order to improve patient outcomes.

Multiple clear cell acanthomas and a sebaceous lymphadenoma presenting in a patient with Cowden syndrome - a case report.

Cowden syndrome (CS) is an uncommon autosomal dominant multiorgan/system genodermatosis. It is characterized by the development of multiple hamartomas of endodermal, mesodermal and ectodermal origin, an increased lifetime risk of breast, thyroid, endometrial and other cancers and an identifiable germline mutation. Mucocutaneous hamartomas are the most common lesions seen and mainly include facial trichilemmomas, oral mucosal papillomas and benign acral keratoses. Herein, we report a case of a 63-year-old Caucasian male with a long-established diagnosis of CS and history of thyroid cancer, colonic polyps, and innumerable trichilemmomas, seborrheic keratoses, squamous papillomas and non-melanoma skin cancers excised in the past. He presented in four separate occasions with small skin-colored papulonodular lesions that upon excision revealed to be clear cell acanthomas. He also developed a tumor in the preauricular area that was completely resected and was found to be a sebaceous lymphadenoma (SLA) of the parotid gland. This is to our knowledge, the second report of clear cell acanthoma and also the second reported case of SLA in a patient with CS.

Linear trichilemmomas on the ankle of a 28-year-old female.

Trichilemmomas are benign cutaneous proliferations derived from the outer root sheath of the hair follicle. They most often occur on the head and neck region and show a female predominance. When multiple, they are associated with Cowden syndrome (CS), a rare disorder due to an autosomal dominant germline mutation in PTEN (phosphatase and tensin homolog on chromosome 10), a tumor suppressor gene. Trichilemmomas outside of the head and neck region are rare, and as such, the association with CS is not clear. A 28-year-old healthy female with no significant family history of cancer presented to her dermatologist with multiple erythematous papules on the left anterior ankle, starting at birth. A shave biopsy confirmed the diagnosis of trichilemmoma with focal desmoplastic features (or desmoplastic trichilemmoma). A PTEN immunohistochemical study showed patchy (but not complete) loss of staining of the lesional cells. After shave removal, the trichilemmomas recurred 1-2 months later.

Simultaneous Development of Three Different Neoplasms of Trichilemmoma, Desmoplastic Trichilemmoma and Basal Cell Carcinoma Arising from Nevus Sebaceus

Nevus sebaceus is a hamartoma of the sebaceous gland that occurs congenitally, from which various secondary tumors can arise with a prevalence of 5%–6%. Benign neoplasms commonly arise from nevus sebaceous, but they have a very low malignant potential. Two neoplasms may occasionally arise within the same lesion, but it is rare for three or more neoplasms to occur in a nevus sebaceus simultaneously. A 61-year-old male patient was admitted to our hospital for a 4 cm×2.5 cm growing tumor in a verrucous form arising within a periauricular nevus sebaceus in the post auricle of the left ear that had developed 30 years earlier. The nodule was diagnosed as 3 different types of tumors: trichilemmoma, desmoplastic trichilemmoma, and basal cell carcinoma. To our knowledge, this is the first report of the coexistence of three different tumors arising from nevus sebaceous. It contain malignant neoplasm also. Surgeons should be aware of the need for close monitoring and early complete surgical excision of sebaceous nevus in order to improve patient outcomes.

Pigmented desmoplastic trichilemmoma.

Desmoplastic trichilemmoma (DT), the pseudomalignant variant of conventional trichilemmoma described by Hunt et al in 1990, displays a superficial lobular growth pattern of glycogen-rich cells with peripheral nuclear palisading surrounded by a thickened basement membrane. DT differs from its conventional counterpart by showing a central hyalinized area with epithelial cords and strands mimicking invasive carcinoma. We report a case that fully satisfies the criteria for DT and, in addition, shows an extensive melanocytic cell component and prominent melanin deposition. To our knowledge, a pigmented variant of DT has not been reported and should be recognized in order to appropriately face the differential diagnosis with malignant pigmented tumors particularly pigmented basal cell carcinoma (BCC).

Trichilemmoma in continuity with pigmented basal cell carcinoma; with dermatoscopy and dermatopathology.

A case of trichilemmoma in continuity with a pigmented basal cell carcinoma is presented with dermatoscopy and dermatopathology. The distinction between the two lesions was evident dermatoscopically and was confirmed dermatopathologically. While trichilemmoma has been reported in association with basal cell carcinoma and dermatoscopy images of four previous cases of trichilemmoma have been published, no previous dermatoscopy image has been published of trichilemmoma associated with basal cell carcinoma.

Anal malignant proliferative trichilemmoma: report of a rare case with review of literature.

Trichilemmoma is a rare type of benign cutaneous neoplasm, which derives from outer sheath of hair follicle. It barely develops malignant progression and has rarely been reported in anal cancer. In this article, we report a case of a 73-year-old woman who presented to the outer-patient department with complaints of a ruptured and longstanding anal phyma. All the appearances were atypical. Blood routine examination showed that neutrophilic granulocyte percentage was elevated and suggest it was a simple inflammation response. No evidence of malignancy was detected upon the laboratory examinations. Then we performed an abscess incision drainage for the patient. A few days later, the biopsy pathological report suggested the specimen is a malignant proliferative trichilemmoma. We decided to perform a wide local excision instead of an extended radical operation in order to preserve anus. After the surgery, we chose not to give chemoradio-treatment for fear of side effects and complications. Careful follow-up indicates that peri-anal malignant proliferative trichilemmoma may have a good prognosis and our treatment is a good choice for the patients with this tumor. Because of the low occurrence rate of anal cancer, especially malignant trichilemmoma, any clinical manifestation and experience are valuable. On one hand, our case may help to take the consideration of the diagnosis of malignant trichilemmoma in case of longtime-suffered peri-anal mass, on the other hand it propose a different treatment method from other anal cancers for clinical doctors.

Human Papillomavirus Deoxyribonucleic Acid may not be Detected in Non-genital Benign Papillomatous Skin Lesions by Polymerase Chain Reaction.

BACKGROUND: Papillomatosis is a known histopathologic pattern usually seen in human papillomavirus (HPV) infection and verruca vulgaris is the typical example. This pattern is also detected in some other benign cutaneous lesions such as nevus sebaceous (NS), seborrheic keratosis (SK), trichilemmoma (TL) and inverted follicular keratosis (IFK). The association between papillomatous lesions and HPV infection is questionable. OBJECTIVE: The objective of this study was to investigate the presence of HPV deoxyribonucleic acid (DNA) in non-genital benign papillomatous skin lesions (NS, SK, TL and IFK) by polymerase chain reaction (PCR). MATERIALS AND METHODS: A total of 100 specimens of non-genital NS, SK, TL and IFK were retrieved from archives of Dermatopathology Department of Razi Hospital, between 2003 and 2010. The conventional PCR using consensus GP5+/GP6+ primer and hydroxymethylbilane synthase gene as inner control was performed. RESULTS: PCR for HPV DNA revealed no positive results in any of 28 seborrheic keratosis (SK), 28 nevus sebaceous (NS), 28 inverted follicular keratosis (IFK) and 13 trichilemmoma (TL) studied specimens. CONCLUSION: Papillomatosis is usually a characteristic pattern of HPV infection. However, we found no association between HPV infection and non-genital benign papillomatous lesions.

A Case of Multiple Eyelid Trichilemmoma Associated with Cowden Syndrome

PURPOSE: To report a case of multiple eyelid trichilemmomas associated with Cowden syndrome. CASE SUMMARY: A 27-year-old woman presented with multiple upper and lower eyelid skin masses that developed over several years. The masses were as large as whitish millet, and were around the upper and lower eyelid margin and the face. The patient had previously undergone subtotal thyroidectomy for a thyroid mass and a mass excision for extremity hemangioma. Excisional biopsy was performed for the diagnosis, and trichilemmoma was diagnosed based on histopathologic examination. Consequently, multiple trichilemmoma associated with Cowden syndrome was diagnosed, and breast evaluations for existence of further masses were recommended. On breast examinations, intraductal papilloma and fibroadenoma were detected. CONCLUSIONS: The trichilemmoma was a hair-follicle benign tumor that also appeared on the skin around the eyelid. If multiple trichilemmoma is diagnosed, an association with Cowden syndrome should be considered as well as presence of masses in other organs.