High-grade trichoblastic carcinoma arising through malignant transformation of trichoblastoma: Immunohistochemical analysis and the expression of p53 and phosphorylated AKT.

Trichoblastoma (TB) is a benign cutaneous adnexal neoplasm. The lesion typically presents as a slow-growing, solitary, well-circumscribed nodule measuring up to 3 cm in diameter. On rare occasions, TB causes malignant transformation into an aggressive form described as high-grade trichoblastic carcinoma. Four such cases have been reported to date; all were described as high-grade trichoblastic carcinomas. Here, we describe the case of a 72-year-old Japanese male patient with a rapidly enlarging subcutaneous tumor on his lower back, which was diagnosed as high-grade trichoblastic carcinoma. Histopathologically, the tumor featured both benign and malignant components, and a transition zone between these states was clearly evident. In the immunohistochemical analysis, a malignant component was positive for p53 and showed stronger staining of phospho-RAC-α serine/threonine-protein kinase (AKT) Ser473 in comparison with a benign component. These results suggest that loss of p53 function and activation of phosphatidylinositol 3-kinase-AKT signaling pathways played important pathogenic roles in malignant transformation of the present case.

Locally aggressive trichilemmal carcinoma.

Trichilemmal carcinoma is a rare adnexal neoplasia with histologic features reminiscent of the outer root sheath of hair follicles. The clinical presentation of trichilemmal carcinoma is variable, as it may mimic basal cell carcinomas and squamous cell carcinomas, thus requiring histologic diagnosis. We report the case of a patient with aggressive trichilemmal carcinoma in the auricle.

Locally aggressive trichilemmal carcinoma

Abstract: Trichilemmal carcinoma is a rare adnexal neoplasia with histologic features reminiscent of the outer root sheath of hair follicles. The clinical presentation of trichilemmal carcinoma is variable, as it may mimic basal cell carcinomas and squamous cell carcinomas, thus requiring histologic diagnosis. We report the case of a patient with aggressive trichilemmal carcinoma in the auricle.

Microcystic Adnexal Carcinoma of the Nipple.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare infiltrative cutaneous tumor, but with its locally aggressive nature, high rate of recurrence, and rare metastatic potential, it is an important clinical entity to consider when faced with a nipple lesion. These tumors are frequently misdiagnosed based on superficial biopsy alone. CASE REPORT: This case of a 15-year-old girl with MAC of the nipple demonstrates the importance of having a high index of suspicion in order to obtain a diagnosis, to determine the extent of disease, and to achieve adequate resection before reconstruction. Close clinical observation is recommended due to the possibility of future recurrences. CONCLUSIONS: Between the confusing nomenclature, indolent course, and inadequacy of superficial biopsy to achieve an accurate diagnosis, MAC is a clinical entity that the plastic surgeon must be aware of in order to facilitate proper diagnosis and treatment.

[Microcystic adnexal carcinoma: A case report]. / Carcinome annexiel microkystique : à propos d'une observation.

We report a case of microcystic adnexal carcinoma in a 80-year-old woman. This is a rare malignant adnexal cutaneous tumor with glandular and follicular differentiation, rare, often asymptomatic, with late diagnosis and slow growth, locally aggressive and rarely metastatic. The distinction with other benign and malignant skin tumors is difficult.

Adnexal Carcinomas Treated With Mohs Micrographic Surgery: A Comprehensive Review.

BACKGROUND: Adnexal carcinomas (ACs) are rare cutaneous malignancies of sweat gland or pilosebaceous origin. Optimal treatment and metastatic potential of AC are poorly defined. Mohs micrographic surgery (MMS) has been increasingly used to treat AC. OBJECTIVE: To review selected primary cutaneous AC and their treatment outcomes with MMS. MATERIALS AND METHODS: Literature review using PubMed search for articles related to primary cutaneous ACs. RESULTS: Sebaceous carcinoma treated with MMS recurred and metastasized in 6.4% and 3.7%, respectively. Primary cutaneous mucinous carcinoma treated with MMS recurred and metastasized in 9.6% and 6.4%, respectively. After MMS, 4.7% of microcystic AC recurred with no reported metastases. After MMS, recurrences and metastases of trichilemmal carcinoma or hidradenocarcinoma have not been reported. Two (4.2%) regional lymph node metastases but no distant metastases or local recurrences have been reported in eccrine porocarcinoma. Squamoid eccrine duct tumor, pilomatrix carcinoma, and spiradenocarcinoma treated with MMS are also reviewed. CONCLUSION: The rarity of ACs and the lack of comparative data on treatment makes conclusive recommendations on treatment difficult. Recent large case series and reviews suggest MMS is a useful and possibly superior treatment for AC and should be considered if primary cutaneous disease is suspected.

[Why is it important to differentiate trichoblastic carcinomas (CT) from basal cell carcinomas (CBC). About 21 cases]. / De l'intérêt de différencier les carcinomes trichoblastiques (CT) des carcinomes basocellulaires (CBC). À propos de 21 cas.

Trichoblastic carcinoma is a rare epithelial malignant epithelial tumor, its diagnosis is difficult and the therapeutic management is non-consensual. This retrospective study, carried out between 2009 and 2015, covered 21 cases and analyzed the diagnostic and therapeutic characteristics of trichoblastic carcinomas. Sex ratio is 2. Trichoblastic carcinoma predominated in the face (65% of cases), particularly in perinasal (30% of cases). Its clinical presentation is in 95% of cases as basal cell carcinoma, which is the first clinical diagnosis evoked. The average size of the tumors was 2.3cm in diameter (from 0.7cm to 15cm). The treatment of these tumors is surgical: the margins retained were on average 0.7cm (0.5cm to 1cm). The first excision was mostly performed under local anesthesia, healthy borders were found in less than 40% of cases, requiring another intervention under general anesthesia with reconstruction by flap or skin graft in nearly 80% of cases. The lymph node metastasis rate was 5%. Three cases of recurrence (17%) occurred between 18 months and 6 years follow-up, despite complete resection. One case recurred three times. These results highlight the difficulty of diagnosing trichoblastic carcinomas, often confused with basal cell carcinomas. Though larger, poorly limited and infiltrating, trichoblastic carcinomas are not really distinguished from basal cell carcinomas. Only the anatomopathological examination of the excision piece make it possible to conclude, the biopsy being most often insufficient. Their local aggressiveness requires a greater margin of excision. The micrographic analysis of Mohs, for the periorificial lesions of the face, would reduce margins, increase their reliability and limit the number of surgical revisions. Finally, the literature reports a high rate of ganglion and visceral metastases (between 9.5 and 11%). Initial search for distal lymph node or metastatic involvement is essential, as well as regular clinical follow-up.

An Aggressive Microcystic Adnexal Carcinoma Infiltrating the Sternum.

Microcystic adnexal carcinoma is a rare cutaneous tumor that is often misdiagnosed and has the potential to be aggressive. Mohs surgery is the treatment of choice to prevent recurrences. We present a case of a large recurrent microcystic adnexal carcinoma on the sternum, initially diagnosed as a basal cell carcinoma. This tumor infiltrated the muscle and bone and was unresectable with Mohs surgery.

J Drugs Dermatol. 2017;16(2):180-181.

Carcinoma triquilemal, um tumor incomum: atualização sobre manejo e prognóstico / Trichilemmal carcinoma, an uncommon tumor: update on its management and prognosis

INTRODUÇÃO: O carcinoma triquilemal (CT) é uma neoplasia rara dos anexos cutâneos. Foi descrito pela primeira vez em 1968, como tricoleptocarcinoma, e tem incidência de 0,05% em pacientes submetidos a exame histopatológico após excisão de lesões cutâneas. Parece ser um tumor de baixa agressividade, porém, relatos na literatura colocam em dúvida tal comportamento indolente. OBJETIVOS: Oferecer uma atualização sobre manejo e prognóstico do CT. MÉTODOS: Pesquisa no PubMed e SciELO com os termos MeSH "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", "carcinoma triquilemal". RESULTADOS: Devido a sua raridade, a maior parte da literatura se baseia em relatos de caso, os quais na sua maioria corroboram a natureza indolente da doença. Excisão cirúrgica é o tratamento preconizado, com baixo risco de recidiva e baixa morbimortalidade. CONCLUSÃO: O comportamento da lesão e as condutas no tratamento do CT baseiam-se em casos isolados ou de pequenas séries de casos. Devido a sua baixa prevalência, uma colaboração multicêntrica agrupando um maior número de casos pode ajudar a definir melhor recomendações de tratamento, fisiopatologia e prognóstico. Excisão cirúrgica continua a ser o padrão-ouro de tratamento, com baixo risco de recidiva.

INTRODUCTION: Trichilemmal carcinoma (TC) is a rare neoplasm of skin appendages. It was first described in 1968 as tricoleptocarcinoma, and has an incidence of 0.05% in patients subjected to histopathological examination after excision of cutaneous lesions. TC has an indolent clinical course ; however, reports in the literature put in doubt this indolent behavior. OBJECTIVES: To provide an update on the management and prognosis of TC. METHODS:A search of the PubMed and SciELO databases by using with the MeSH terms "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", and "carcinoma triquilemal" was performed. RESULTS: Owing to the rarity of TC, most studies were case reports, which essentially corroborate the indolent nature of the disease. Surgical excision is the recommended treatment, and is associated with a low risk of recurrence and low morbidity and mortality. CONCLUSION: The behavior of the lesion and the procedure of treatment of TC are based on isolated cases or in a small series of cases. Because of its low prevalence, a multicenter collaboration of a greater number of cases can help define the best treatment recommendations, pathophysiology, and prognosis. Surgical excision remains the gold standard of treatment, and is associated with a low risk of recurrence.

Carcinoma ductal ecrino: papel de la cirugía de Mohs y revisión de la literatura / Eccrine Carcinoma: The Role of Mohs Micrographic Surgery and a Review of the Literature

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Focus of tricholemmal differentiation (tricholemmal carcinoma) within Bowen's disease/carcinoma.

Bowen's disease (BD)/carcinoma is a type of squamous cell carcinoma of the skin, however, the possibility of adnexal differentiation (the development of sebaceous carcinoma or porocarcinoma) occurring in BD/carcinoma has been suggested. We herein describe a case of BD with superficial invasive carcinoma, which showed a clear cell focus, demonstrating tricholemmal differentiation. This clear cell focus showed the following findings: primarily composed of clear cells, somewhat columnar clear cells aligned in a palisade along a discernible basement membrane, tricholemmal keratinization and glycogen contained within the cells. In addition, the immunohistochemical profile in this clear cell focus, namely, negative staining for cytokeratin (CK)1 and positive staining for CK17 and calretinin in the inner cells of the neoplastic lobule, corresponded to that of the outer root sheath cells. This case suggested that adnexal differentiation can rarely occur within true BD/carcinoma, although adnexal carcinomas are commonly associated with a simple bowenoid change.

Microcystic adnexal carcinoma simulating scarring alopecia.

The microcystic adnexal carcinoma is a rare, locally aggressive malignant adnexal neoplasm associated with significant morbidity. It is often underdiagnosed due to clinical and histopathological resemblance with other cutaneous neoplasms and/or a combination of lack of familiarity associated with inadequate samples. We report a case with clinical hypothesis of scarring alopecia and histopathological diagnosis of microcystic adnexal carcinoma with favorable outcome in a follow-up of eleven years, after surgical treatment.