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.

Carcinoma cutáneo de células de Merkel, a propósito de un caso y revisión de la literatura / Cutaneous carcinoma of merkel cells, purpose of a case and review of literature

El carcinoma de células de Merkel o también llamado carcinoma neuroendocrino cutáneo es una neoplasia muy rara y agresiva. Se caracteriza por su tendencia a la recurrencia, diseminación ganglionar y metástasis a gran distancia. Se presenta el caso de un paciente masculino de 88 años de edad, con antecedente de carcinoma baso celular en región dorsal, que presenta hace 3 meses una tumoración cutánea en tobillo derecho de gran tamaño, que produce metástasis ganglionar a distancia, el estudio de inmunohistoquímica revela que se trata de un carcinoma neuroendocrino cutáneo, se realiza excéresis del mismo con bordes histológicos negativos, pero a los pocos meses recidiva por lo que se realiza cirugía más profunda y se acompaña de quimioterapia. Se pone énfasis en lo agresivo y tendencia a la recidiva de esta rara enfermedad que podría pasar desapercibida en centros de menor complejidad.

The Merkel cell carcinoma, also known as cutaneous neuroendocrine carcinoma, is a very rare and aggressive neoplasm. It is characterized by its tendency to recurrence, lymph node dissemination and distant metastasis. We present the case of a 88-year-old male patient with a history of basal cell carcinoma in the dorsal region, who presented a large right cutaneous tumor in the right ankle 3 months ago, which produces distant lymph node metastasis. The immunohistochemical study reveals that it is a cutaneous neuroendocrine carcinoma, exeresis is performed with negative histological edges, but after a few months it relapses, so a deeper surgery is performed and it is accompanied by chemotherapy. Emphasis is placed on the aggressiveness and tendency to recurrence of this rare disease that could go unnoticed in centers of less complexity.

Ductal eccrine carcinoma of the axilla: a diagnostic pitfall

Abstract: Ductal eccrine carcinoma (DEC) is a rare sweat gland carcinoma with ductular differentiation. Clinically, it is characterized by a slowly growing, hardened plaque or nodule predominantly located on the head and neck. Histologically, DEC shares similar features to invasive breast carcinoma, thus causing great diagnostic challenges. We report a 69-year-old woman who presented with a hardened plaque on the axilla. A skin biopsy was performed and metastatic invasive breast carcinoma could not be ruled out. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

A case of eccrine carcinoma presenting with neurological manifestations

INTRODUCTION: Eccrine  carcinoma  is  an  extremely  rare  skin tumor where only 1/13000 specimens have been submitted to dermatopathological  laboratories  in  the  United  States.There  is  no  data  yet  to compare the Philippines with the international  incidence  of  eccrine  carcinoma.  This is a case of a 69-year-old Filipino female who presented with a recurring invasive indolent tumor at the right fronto-parietal area who presented with left sided hemiparesis and seizure.CASE: The patient was presented with a recurrent invasive indolent  mass  on  her  right  front-parietal  area,  grossly measuring five by four centimeters, nodular flesh colored,which  extended  intracranially.This was associated with left sided hemiparesis and due to the extent of the tumor encroaching through the brain parenchyma, patient was noted  to  have  seizure  episodes.  The  patient  was  given surgical and radiologic options however, she did not comply and died last December 2015.RESULTS: A cranial MRI with MRA showed a  heterogenous enhancing intracranial mass with extracranial component with compressed entrapped and depressed superior sagittal sinus  by  the  axial  mass  witin  calvarial  penetration  and scalp involvement compressing on the right parietal lobe with parenchyma edema. Biopsy was eventually done and findings were consistent with an eccrine carcinoma.CONCLUSION: This is the first case of eccrine carcinoma in our institution. Due to the paucity of data, there are no guidelines to the management of an eccrine carcinoma. Hence the imperative need to raise awareness regarding this rare tumor because,  without  a  high  index  of  suspicion  this  rare  entity may be overlooked or misdiagnosed. When presented with an indolent invasive recurrent tumor a high index of suspicion that  an  eccrine  Carcinoma  may  be  suspected.Excision biopsy may be done for correct identification of the tumor.

A case of eccrine carcinoma presenting with neurological manifestations

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Eccrine  carcinoma  is  an  extremely  rare  skin tumor where only 1/13000 specimens have been submitted to dermatopathological  laboratories  in  the  United  States.There  is  no  data  yet  to compare the Philippines with the international  incidence  of  eccrine  carcinoma.  This is a case of a 69-year-old Filipino female who presented with a recurring invasive indolent tumor at the right fronto-parietal area who presented with left sided hemiparesis and seizure.<br /><strong>CASE:</strong> The patient was presented with a recurrent invasive indolent  mass  on  her  right  front-parietal  area,  grossly measuring five by four centimeters, nodular flesh colored,which  extended  intracranially.This was associated with left sided hemiparesis and due to the extent of the tumor encroaching through the brain parenchyma, patient was noted  to  have  seizure  episodes.  The  patient  was  given surgical and radiologic options however, she did not comply and died last December 2015.<br /><strong>RESULTS:</strong> A cranial MRI with MRA showed a  heterogenous enhancing intracranial mass with extracranial component with compressed entrapped and depressed superior sagittal sinus  by  the  axial  mass  witin  calvarial  penetration  and scalp involvement compressing on the right parietal lobe with parenchyma edema. Biopsy was eventually done and findings were consistent with an eccrine carcinoma.<br /><strong>CONCLUSION:</strong> This is the first case of eccrine carcinoma in our institution. Due to the paucity of data, there are no guidelines to the management of an eccrine carcinoma. Hence the imperative need to raise awareness regarding this rare tumor because,  without  a  high  index  of  suspicion  this  rare  entity may be overlooked or misdiagnosed. When presented with an indolent invasive recurrent tumor a high index of suspicion that  an  eccrine  Carcinoma  may  be  suspected.Excision biopsy may be done for correct identification of the tumor.</p>

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.

Microcystic adnexal carcinoma simulating scarring alopecia

The microcystic adnexal carcinoma is a rare, locally aggressive malignant adnexal neoplasm associated with signifi cant 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.

Large cutaneous apocrine carcinoma occurring on right thigh aggravated after moxa treatment

Primary cutaneous apocrine carcinoma is a rare adnexal tumor of the skin that occurs mainly in the axilla, anogenital area while the scalp and the lower extremities, especially the thigh, are very unusual sites. However, clinical or pathologic characteristics have not been well established due to a paucity of this tumor. Herein, we report very unusual case of apocrine carcinoma present as a huge mass in the lateral thigh of 77-year-old woman, which was aggravated abruptly after an irritation by moxa treatment, with a brief review of the literature.

Epidermodysplasia verruciformis: An unusual malignant transformation.

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Trichilemmal Carcinoma of the Upper Eyelid: A Case Report

We report a very rare case of trichilemmal carcinoma (TLC) involving the upper eyelid. To the best of our knowledge, this is the first report of trichilemmal carcinoma of the upper eyelid in Korea. A 51-year-old man presented to our hospital complaining of a bloody discharge from his left upper eyelid. He had a soft and lobulated mass on the palpebral conjunctiva. An incisional biopsy revealed trabecular growth of tumor cells with clear cytoplasm, prominent nucleoli, frequent mitoses, and foci of trichilemmal keratinization. Immunohistochemically, the lesion was positive for p53 and negative for CD 34. A diagnosis of TLC was made, and total excision of the mass and reconstruction of the eyelid were performed. Trichilemmal carcinoma is a rare malignant tumor, though it appears to be an indolent neoplasm with no metastatic potential. The treatment of choice for trichilemmal carcinoma of the eyelid is complete excision with tumor-free margins due to the locally invasive nature of the lesion.

Sweat gland carcinoma with lung metastases.

Sweat gland carcinoma is a rare skin tumor. The tumor has propensity to spread to lymph nodes and distant metastases has been reported. Their exact incidence in the Indian setting is not known. Aspects related to treatment are also not clearly defined. Though surgery forms the initial treatment approach, adjuvant treatment has not been properly explored. We report here a case of sweat gland carcinoma with bilateral lung metastases.

Metástasis de carcinoma epidermoide a la glándula mamaria: reporte de un caso y revisión de la literatura / Metastasis of epidermoid carcinoma to the mammary gland

Se presenta el caso de una paciente de 63 años de edad con antecedente de quemadura en miembro superior derecho durante la primera infancia, sin otros datos de importancia. Presenta un tumor ulcero vegetante localizado en la cicatriz de la quemadura de un año de evolución, de 22 x 15 cm, fijo a planos profundos, acompañado de sangrado y adenomegalias axilares, histocitopatología demostró un carcinoma epidermoide moderadamente diferenciado. En una revisión de rutina, seis meses subsiguientes a su tratamiento, se descubre un nódulo en el cuadrante superior externo de la glándula mamaria derecha, de 2 cm de diámetro, duro, móvil, irregular y no doloroso. La mastografía demostró una lesión nodular; de alta densidad, de contorno irregular, sin microcalcificaciones; el ultrasonido mamario confirmó una lesión sólida. Se estableció el diagnóstico de carcinoma epidermoide mamaria Etapa clínica 1 por TI No Mo.

Squamous cell carcinoma in situ arising in mature cystic teratoma.

Mature cystic teratomas of ovary are common tumors. A few may exhibit malignant degeneration, most of these being invasive squamous cell carcinoma. We report a rare case of pure squamous cell carcinoma in situ in benign cystic teratoma.

Piloleiomyoma--a report of five cases.

Piloleiomyomas are uncommon smooth muscle neoplasms of the skin with a few reproted cases in Indian literature (1,2,3,4,5). They are often misdiagnosed clinically. A correct biopsy report is important because patients may have to be managed medically since surgery is associated with a high rate of recurrence. The classical histologic findings, and Masson's stain to confirm the smooth muscle origin aids in the correct diagnosis.

Tumores carcinoides / Carcinoid tumors

La organización Mundial de la Salud (OMS) definió todos los tumores carcinoides integrales del sistema neuroendocrino excepto el cáncer (Ca) medular del tiroides, Ca de células pequeñas del pulmón, paragangliomas, tumor de Merckel de la piel, tumor de islotes pancreáticos, adenomas pituitarios y feocracitoma.

Carcinoma anexial microquístico: tratamiento exitoso con extirpación quirúrgica amplia / Microcystic adnexal carcinoma: successfull treatment with wide surgical excision

El carcinoma anexial microquístico, también llamado carcinoma esclerosante de conductos sudoríparos, fue descripto por primera vez por Goldstein y cols. en 1982. Desde entonces varias presentaciones de este tumor han ayudado a definir sus características. Nosotros publicamos un caso de presentación clínica e histopatológica típica y hacemos una revisión de la literatura poniendo énfasis en el tratamiento que en nuestro caso se efectuó mediante la extirpación quirúrgica amplia de la lesión

Poroma ecrino maligno / Malignant eccrine poroma

Presentamos una paciente de 90 años de edad, sexo femenino, que desarrolla un poroma ecrino maligno en cara anterior de muslo izquierdo. Consideramos de interés la presentación efectuada dada la rareza de estos carcinomas y realizamos una reseña de las características clínicopatológicas, hallazgos ultramicroscópicos e inmunohistoquímicos y conducta biológica de estos tumores