Mohs micrographic surgery in rare cutaneous tumors: a retrospective study at a Brazilian tertiary university hospital

Abstract Background Mohs micrographic surgery is an established technique in the treatment of cutaneous neoplasms. It offers higher cure rates and the main indications are non-melanoma malignant skin tumors. Few studies have been performed on the treatment of rare tumors through this technique. Objective To study rare skin tumors and rare variants of basal cell carcinoma and squamous cell carcinoma submitted to Mohs micrographic surgery in a tertiary service in relation to frequency, disease-free evolution, and applicability of this surgical procedure for this group of tumors. Methods This was a retrospective observational study including rare skin tumors and less common variants of basal cell carcinoma and squamous cell carcinoma treated using Mohs micrographic surgery, between October 2008 and April 2021. Results During the study period, 437 tumors were treated using Mohs micrographic surgery, and 22 (5%) rare skin tumors were selected. The tumors comprised three dermatofibrosarcomas protuberans, two atypical fibroxanthomas, two spiradenomas, two hypercellular fibrohistiocytomas, one primary cutaneous adenocarcinoma, one trichoblastoma, one porocarcinoma, one chondroid syringoma, one cutaneous angiosarcoma, one Merkel cell carcinoma, and one sebaceous carcinoma. Six other cases of rare basal cell carcinoma variants with trichoepitheliomatous differentiation, metatypical basal cell carcinoma, and clear cell squamous cell carcinoma were included. There were no cases of recurrence after an average of six years of follow-up. Study limitations This is a retrospective study on rare neoplasms carried out in a single referral center, and this surgical technique isn't widely available in the public service. Conclusion This retrospective case series showed that Mohs micrographic surgery is an appropriate treatment for rare skin tumors. They corresponded to 5% of the tumors treated by the technique during a 12-year-period, with no recurrences identified.

Hidradenoma nodular maligno / Malignant Nodular Hidradenoma

Introducción: El hidradenoma nodular maligno es un tumor maligno de glándula sudorípara ecrinas, poco común, considerada una lesión de diferenciación anexial ecrinas, que generalmente surge de nuevo, aunque se han descrito unos pocos casos surgidos sobre un hidradenoma nodular. Es decir, representa la contrapartida maligna del hidradenoma nodular. Objetivo: Dar a conocer la presentación de un caso, dada la inusual aparición de esta entidad, con revisión de los criterios para su diagnóstico. Caso clínico: Se informa el caso de un hombre de 74 años de edad con una neo formación en la región parietal derecha del cuero cabelludo. Conclusiones: Debemos pensar en un hidradenoma nodular maligno ante un tumor solitario, firme o fluctuante, infrecuente en el cuero cabelludo, con curso agresivo, recurrencias y metástasis ganglionares y confirmar su diagnóstico con el estudio inmunohistoquímico(AU)

Introduction: Malignant nodular hidradenoma is a rare malignant eccrine sweat gland tumor considered a lesion of eccrine adnexal differentiation, which usually arises again, although a few arising cases on nodular hidradenoma have been described. In other words, it represents the malignant counterpart of nodular hidradenoma. Objective: To report a case, given the unusual occurrence of this entity, with a review of the criteria for its diagnosis. Case report: We report the case of a 74-year-old man with a neoformation in the right parietal region of the scalp. Conclusions: We should consider a malignant nodular hidradenoma when faced with a solitary, firm or fluctuant tumor, rare in the scalp, with aggressive evolution, recurrences and lymph node metastasis, and confirm its diagnosis with immunohistochemical study(AU)

Perianal hidradenoma papilliferum / Hidradenoma papilífero perianal

ABSTRACT Hidradenoma papilliferum is a benign cystic tumor originated in apocrine sweat glands of the anogenital region. It is common in vulvar topography and rare in the perianal region. A well-documented case of a patient with slow-growing perianal nodulation without pain or other symptoms is reported. The histological study shows a hidradenoma papilliferum. The article reviews the topic and discusses the importance of the differential diagnosis of proctological conditions. A biopsy is mandatory in anal lesions of unusual presentation.

RESUMO O hidradenoma papilífero é um tumor cístico benigno originado nas glândulas sudoríparas apócrinas da região anogenital, sendo comum em topografia vulvar e raro na região perianal. Relata-se um caso bem documentado de paciente com nodulação perianal de crescimento lento, sem dor ou outros sintomas, cujo estudo histológico revelou se tratar de hidradenoma papilífero. O artigo revisa o tema e discute a importância do diagnóstico diferencial das afecções proctológicas, sendo a biópsia obrigatória em lesões anais de apresentação não-usual.

Case for diagnosis. A bluish nodule on the scalp

Abstract: Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Porocarcinoma: relato de caso / Porocarcinoma: case report

O porocarcinoma écrino é uma neoplasia maligna, rara, das glândulas sudoríparas écrinas e, com maior frequência, acomete os indivíduos idosos, com idade média de 67,5 anos. Apresenta manifestações clínicas variadas e sua localização não se correlaciona com a concentração das glândulas sudoríparas, ocorrendo, principalmente, nos pés e pernas. O seu diagnóstico e tratamento devem ser precoces para evitar a agressividade do tumor, que ocorre em 20 por cento dos casos. Os autores relatam o caso de um porocarcinoma extenso no tórax, em paciente do sexo masculino, de 71 anos.

Eccrine porocarcinoma is a rare, malignant neoplasm of eccrine sweat glands. It often occurs in elderly people, mean age of 67.5 years. The clinical features are variable and their localization is not related to sweat-gland concentration, occurring mainly on the feet and legs. Disease diagnosis and treatment should be as early as possible in order to avoid tumor aggressiveness that occurs in 20 percent of the cases. The authors present a case of extensive porocarcinoma on the thorax of a 71-year-old male patient.

Porocarcinoma écrino: relato de quatro casos e revisão da literatura / Eccrine porocarcinoma: report of four cases and literature review

Porocarcinoma écrino é um tumor de pele raro derivado do acrossiríngeo. Quatro casos de porocarcinoma écrino são estudados. Os pacientes tinham idade entre 49 e 64 anos, sendo dois homens e duas mulheres. O tempo de crescimento dos tumores variou de um a 20 anos. As lesões mediam de 1,2 x 2 a 4 x 5 cm e se localizavam nas extremidades ou na face. Dois pacientes apresentavam carcinoma basocelular em sítios diferentes do porocarcinoma écrino. Duas das biópsias foram previamente descritas como poroma écrino; entretanto, malignidade foi observada em todos os casos após excisão ampla. Portanto, em toda biópsia de pele compatível com poroma écrino, deve-se considerar a possibilidade de malignidade.

Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.

Fine Needle Aspiration Cytology of Eccrine Acrospiroma of the Breast: Report of a Case Misdiagnosed as Ductal Carcinoma / 대한세포병리학회지

Eccrine acrospiroma is a rare adnexal tumor of the skin. When the clinical presentation is that of a breast lump, diagnosis can be difficult. Also, most of the cytopathologists are not familiar with the cytologic features of this tumor and this is responsible for diagnostic pitfalls. We experienced a case of eccrine acrospiroma of the right breast in a 41-year-old female, misdiagnosed by fine needle aspiration cytology(FNAC). FNAC was characterized by tight clusters or sheets of small round cells, polygonal cells, and spindle cells and tubule like structures within clusters. Myoepithelial cells were not noted in the clusters. The diagnosis of eccrine acrospiroma was confirmed by histology.

A Case of Eccrine Acrospiroma in Scrotum / 대한비뇨기과학회지

Acrospiroma is a rare benign tumor and a clinicopathologically distinctive neoplasm differentiating toward the distal part of the eccrine sweat gland. I experienced a case of dermal acrospiroma in a 59-year-old man who had a 7.0x6.5 x 5.3 cm sized, slowly growing, brown colored, smooth-surfaced mass on the right scrotum for 3 years. Histological examination revealed lobular structures composed of small fusiform cells and large eosinophilic polyhedral cells. Histochemically some of large polyhedral cells had PAS-positive DPAS-negative glycogen and Alcian-positive mucin in cytoplasm. Immunohistochemical stainings for vimentin, S-100, low molecule keratin, and EMA were positive in the tumor cells. During 6 months follow up period after excision, there was no recurrence. However, it is necessary to follow up for a long period because some acrospiroma may recur after a long silence period.

A Case of Cutaneous Cholesterol Embolism / 대한피부과학회지

Cutaneous cholesterol embolism is a disease with severe arterosclercsis of the abdominal aorta in which emboli of cholesterol crystals from atheromatous plaques occlude airterioles. We report a case of cutaneous cholesterol embolism in a 61-year-old man who had livedo reticularis on both his feet for 10 days. Histopathologic findings showed needle-shaped cholesterol clefts viithin the lumina of arterioles. The patient died of myocardial infarction 3 months later.

Malignant Eccrine Acrospiroma: A case report

A case of malignant eccrine acrospiroma of the anterior chest wall is reported. This mass had been present for 20 years and during rescent 5 years it showed frequent ulceration and bleeding suggesting malignant transformation. Microscopically, several foci malignant transformation from preexisting benign eccrine acrospiroma are seen and in immunohistochemical staining, the tumor cells both benign and malignant portion, show positive reaction to cytokeratin.

A case of acrospiroma / 대한피부과학회지

A healthy 12-year-old girl had noted over 2 years a gradual-growing painless nodule on the area below the right axilla. The lesion measuring approxima:;ely 1.2 x 1.2 x 0.4cm in size, had a reddish-blue colored ulcerative papule raised on its cente with mucous discharge to compression. Histopathelogically, a circumscribed solid tumor located in the deep dermis, revealed the predominnntly epidermoid features of polyhedral cells, with the mucous cells within and at the peripiiery of the tumor. The mucous cells exhibited diastase-resistant PAS -positive, alcian-blue reactive at pH 2.5 and mucicarmine-positive reactions. Results of irnmunoperoxidase staining for cytokeratin and carcinoernbryonic antigen were focally cytoplasmic-positive, while negative for S-100 protein and epithlial membrane antigen. She has remained free of disease one year following excision.