Lesiones metastásicas a glándula tiroides. Una serie de casos / Metastatic lesions to the thyroid gland. Case series

Las lesiones metastásicas representan hasta un 3 % de los tumores malignos de la glándula tiroides. La mayoría de los casos se originan de tumores de células renales y de pulmón. El abordaje diagnóstico implica una alta sospecha clínica en pacientes con primarios conocidos, sin embargo, puede ser la manifestación inicial de una enfermedad maligna extensa no diagnosticada hasta en un 20 % a 40 % de los pacientes. La biopsia por aguja fina ha demostrado buen rendimiento para el diagnóstico de los nódulos metastásicos. El pronóstico y la opción del tratamiento quirúrgico dependen del control local del primario y del estado de la enfermedad sistémica asociada, por lo tanto, debe ser individualizado. Por lo general, hasta un 80 % de los pacientes con compromiso de la tiroides tienen enfermedad metastásica multiorgánica, y la intención del tratamiento quirúrgico es con fines paliativos para prevenir las complicaciones derivadas de la extensión local de la enfermedad a las estructuras del tracto aerodigestivo superior en el cuello. Se presenta a continuación, una serie de seis casos de pacientes con lesiones metastásicas a glándula tiroides con primarios en riñón, mama y de melanomas

Metastatic lesions represent up to 3% of malignant tumors of the thyroid gland. Most cases originate from lung and renal cell tumors. The diagnostic approach implies a high clinical suspicion in patients with known primaries, however, it can be the initial manifestation of an extensive undiagnosed malignant disease in up to 20% to 40% of patients. Fine-needle biopsy has shown good performance for the diagnosis of metastatic nodules. The prognosis and the option of surgical treatment depend on the local control of the primary condition and the state of the associated systemic disease, therefore it must be individualized. In general, up to 80% of patients with thyroid involvement have multi-organ metastatic disease and surgical treatment is intended to be palliative to prevent complications resulting from local extension of the disease to structures of the upper aerodigestive tract in the neck. A case series of six patients with metastatic lesions to the thyroid gland with primaries in the kidney, breast and melanomas is presented below

Tricoepitelioma múltiple familiar: a propósito de un caso / Familial multiple trichoepithelioma: a case report

El Tricoepitelioma Múltiple Familiar (TMF) constituye una rara enfermedad autosómica dominante, se caracteriza por la aparición de múltiples pápulas color piel, monomorfas, simétricas, ubicadas en la región central de la cara. El diagnóstico es histopatológico, donde se encuentran tricoepiteliomas, los cuales son neoplasias anexiales benignas que se originan en los folículos pilosos. La condición es de comportamiento indolente, pero con una importante repercusión estética y de difícil manejo. Al ser esta una entidad poco frecuente, el objetivo de este artículo es actualizar los aspectos más relevantes de esta enfermedad. Se presenta el caso de una paciente de 23 años con lesiones faciales típicas en quien se confirmó el diagnostico de TMF

Familial Multiple Trichoepithelioma (FMT) is a rare autosomal dominant disease, characte-rized by the appearance of multiple papules of skin color, monomorphic, symmetrical and located in the central region of the face. The diagnosis is based on histopathological features of trichoepitheliomas, which are benign adnexal neoplasms that originate in the hair follicles. The condition has an indolent behavior but it has an important aesthetic repercussion and it's difficult to treat. As this is a rare entity, the objective of this article is to update the most relevant aspects of this disease. We present the case of a 23 year old patient with typical facial lesions in whom the diagnosis of FMT was confirmed.

Cutaneous leiomyosarcoma on the face

Abstract: Leiomyosarcoma is a rare skin tumor, most common in white men in the fifth to eighth decades of life. Primary tumors are classified in dermal or subcutaneous, that differ by clinical and prognostic features. They may appear on any site of the body, but are rare on the face. A 54-year-old female was admitted with a 5cm exophytic nodular lesion of 8 months duration on the right cheek, site of previous chronic radiodermatitis. Histopathology revealed spindle-shaped cell neoplasia, positive for smooth muscle actin on immunohistochemistry. Cutaneous leiomyosarcomas on the face are rare and may occur in previously irradiated areas. Immunohistochemistry is mandatory for an accurate diagnosis. Its similarity with other tumors may complicate the diagnosis, with delay expansion of the tumor.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

The prognostic importance of parotid involvement by head and neck squamous cell carcinoma - Case report

Abstract Squamous cell carcinoma (SCC) is the second-most common malignant cutaneous cancer, with 60% occurring in the head and neck region. Metastases are uncommon and imply a more conservative prognosis. This report describes a case of parotid-invasive, facial squamous cell carcinoma, highlighting the importance of its prognostic and therapeutic management. The patient is an 81-year-old female, exhibiting extensive tumoral lesions in the pre-auricular region, affecting the parotid parenchyma and implying the metastatic involvement of the intra-parotid lymph node. Parotid involvement caused by SCC in specificity tumors is discussed herein. Parotid invasion is currently recognized as an isolated variable. It affects survival rates and determines certain changes in case management, such as the broadening of resection areas and adjuvant radiotherapy.

Oral and Maxillofacial Complex Lesions in Adolescents: A Retrospective Study of 20 Years / Lesiones Orales y del Complejo Maxilofacial en Adolescentes: Un Estudio Retrospectivo de 20 Años

Studies assessing the prevalence of oral lesions show that 5.2% to 12.8% of biopsy specimens are from children and adolescents. In Brazil, there are few studies analyzing the distribution of oral lesions in that population. Therefore, the aim of this study was to evaluate the distribution of oral and maxillomandibular lesions of adolescents, admitted to the Department of Oral Diagnosis of the Federal University of Paraná (UFPR) from 1994 to 2013. A study based on the review of the charts was done and the lesions were classified in 11 categories: salivary glands disease, dental pathology, gingival and periodontal pathology, odontogenic cysts, odontogenic tumors, non odontogenic cysts, bone pathology, mucosal pathology, connective tissue pathology, malignant tumors and other pathology. Variables including age, sex, ethnicity, diagnosis and lesion location were also evaluated. Upon analysis, 376 lesions were identified, most in girls, 51.9% and 77.1 % of patients were white. The most frequent site was the lower lip followed by the gingiva. Mucocele was the most common lesion (27.6%), followed by fibroepithelial hyperplasia (8.2%) and pyogenic granuloma (5.3%). Although there is a wide range of pathologies that can occur in the oral cavity, traumatic injuries are the most common in this age group.

Los estudios que evalúan la prevalencia de lesiones orales muestran que entre el 5,2% hasta el 12,8% de las muestras de biopsia corresponden a niños y adolescentes. En Brasil, hay pocos estudios que hayan analizado la distribución de las lesiones orales enesa población. Por lo tanto, el objetivo de este estudio fue evaluar la distribución de las lesiones orales y maxilofaciales de adolescentes, ingresados en el Servicio de Diagnóstico Oral de la Universidad Federal de Paraná (UFPR) entre 1994 a 2013. Se llevó a cabo la revisión de historias clínicas y las lesiones halladas fueron clasificadas en 11 categorías: enfermedad de glándulas salivales, patología dental, patología gingival y periodontal, quistes odontogénicos, tumores odontogénicos, quistes no odontogénicos, patología ósea, patología de la mucosa, patología del tejido conectivo, tumores malignos y otras patologías. También se evaluaron otras variables como edad, género, etnia, diagnóstico y localización de la lesión. Luego del análisis, se identificaron 376 lesiones, la mayoría en niñas (51,9%), siendo el 77,1% de los pacientes de raza blanca. La localización más frecuente fue el labio inferior seguida de la encía. El mucocele fue la lesión más común (27,6%), seguido de hiperplasia fibroepitelial (8,2%) y granuloma piógeno (5,3%). Aunque existe una amplia serie de patologías que pueden ocurrir en la cavidad oral, las lesiones traumáticas son las más comunes en este grupo de edad.

Contoured technique for lentigo maligna

The surgical approach to lentigo maligna is a challenge to dermatologists, given the difficulty of clinical delimitation of borders. We report here a case of a 69-year-old female patient presenting with brownish macules on her face, since 10 years ago, with histopathological diagnosis of lentigo maligna. The surgical management employed was excision of visible borders with the contoured technique and immediate submission of these borders for histopathological analysis before complete excision of the tumor. This technique is a variant of staged excision, with lower rates of recurrence and acceptable aesthetic results.

Malformación linfática facial mixta: Caso clínico / Mixed facial lymphatic malformation: Case report

Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions. Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up. Case report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension. Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

El linfangioma corresponde a una malformación vascular de los vasos linfáticos, preferentemente de la región de cabeza y cuello. La mayoría de los casos son de evolución progresiva y requieren un manejo multidisciplinario. Actualmente la primera opción terapéutica es la esclerosis, reservando la cirugía para el tratamiento de las lesiones remanentes. Objetivo: Presentar un caso de malformación linfática (ML) facial, tratado con escleroterapia, cirugía y ortodoncia en un seguimiento a 15 años. Caso clínico: Paciente de sexo femenino que consulta al año de edad por aumento de volumen progresivo de partes blandas en su mejilla derecha. El estudio de imágenes confirmó el diagnóstico de Malformación Linfática microquística. Se manejó con esclerosis seriada con OK-432 y Bleomicina. A los 2 años de edad se consideró que la respuesta era adecuada, y se procedió a realizar extirpación quirúrgica intralesional submandibular, con resección parcial de la lesión. La biopsia confirmó el diagnóstico de ML microquística. Seis meses después se planificó nueva resección utilizando el mismo abordaje y extirpando lesión remanente, con evolución favorable hasta la edad de 9 años en que requiere cirugía y manejo por ortodoncia, por recidiva de lesión a nivel intraoral. Evolución favorable hasta que a la edad de 13 años se planifica nueva cirugía y manejo por ortodoncia para suspender la comisura bucal derecha. Conclusión: El manejo de la ML mediante escleroterapia, cirugía, y ortodoncia muestra en este caso las ventajas de un tratamiento multidisciplinarion a largo plazo.

Extra ocular sebaceous adenocarcinoma in HIV-positive patient - case report / Adenocarcinoma sebaceo extra-ocular em paciente HIV positivo - relato de caso

Sebaceous adenocarcinoma is a rare adnexal tumor that can affect the skin and is divided into ocular, a more common form and extra ocular, of a rarer occurrence. We report the case of a patient diagnosed with Acquired Immune Deficiency Syndrome (AIDS) who developed an extra ocular, bulky and fast-growing sebaceous adenocarcinoma on the face. The literature has suggested that transplanted patients and HIV-positive patients have an excess risk for developing adnexal tumors, including sebaceous adenocarcinoma.

Adenocarcinoma sebáceo é um tumor anexial raro que pode envolver a pele e é dividido em ocular, mais comum e extraocular, mais raro. Relatamos o caso de um paciente com diagnóstico de Síndrome da Imunodeficiência Adquirida que desenvolveu um adenocarcinoma sebáceo extra-ocular, na face, volumoso, de rápido crescimento. A literatura tem sugerido que pacientes transplantados e portadores do vírus da imunodeficiência humana têm um excesso de risco para o desenvolvimento de tumores anexiais, incluindo o Adenocarcinoma sebáceo.

Desmoplastic melanoma associated with an intraepidermal lentiginous lesion: case report and literature review / Melanoma desmoplásico associado a lesão lentiginosa intraepidérmica, com evolução de 10 anos: relato de caso e revisão bibliográfica

Desmoplastic melanoma tends to present as firm, amelanotic papules. Microscopically, it reveals a proliferation of fusiform cells in the dermis and variable collagen deposition, as well as intraepidermal melanocytic proliferation of lentiginous type in most cases. Biopsy in a 61-year-old white male patient, who had received a diagnosis of lentigo maligna on his face 10 years before, revealed a proliferation of dermal pigmented spindle cells and collagen deposition, reaching the deep reticular dermis, with a lentiginous component. Immunohistochemistry with S-100, Melan-A and WT1 showed positivity, but it was weak with HMB45. Desmoplastic melanoma associated with lentigo maligna was diagnosed. Several authors discuss whether desmoplastic melanoma represents a progression from the lentiginous component or arises "de novo". Desmoplastic melanoma represents a minority of cases of primary cutaneous melanoma (less than 4%). Identification of lentigo maligna indicates that desmoplastic melanoma should be carefully investigated.

Os melanomas desmoplásicos apresentam-se como pápulas amelanóticas firmes; à microscopia exibem proliferação de células fusiformes na derme e variável deposição de colágeno, além de proliferação melanocítica lentiginosa, intraepidérmica, na maioria dos casos. Realizada biópsia de pele de paciente masculino, 61 anos, branco, com diagnóstico de lentigo maligno na face, há 10 anos. O exame histopatológico revela proliferação dérmica de células fusiformes pigmentadas e deposição de colágeno, invadindo até a profundidade da derme reticular, associado a componente lentiginoso; presença de positividade imuno-histoquímica com S-100, Melan-A e WT1, e marcação fraca com HMB45. Diagnóstico de melanoma desmoplásico, associado a lentigo maligno. Existe divergência quanto à origem do melanoma desmoplásico, a partir do componente lentiginoso ou "de novo", na ausência de lentigo associado. O melanoma desmoplásico representa uma minoria dos casos de melanoma cutâneo primário (menos de 4%). A presença de lentigo maligno pode servir de sinal de alerta para possível relação com melanoma desmoplásico.

Lesiones proliferativas más frecuentes del complejo bucomaxilofacial / Most frequent proliferative lesions of the Buco Maxillofacial Complex

Introducción: las lesiones proliferativas son entidades que se presentan en la cavidad bucal. Algunas son de origen traumático y otras son neoplasias. Con frecuencia no son bien diagnosticadas en la clínica, por la similitud que pueden presentarse entre ellas. Objetivo: determinar las características de las lesiones proliferativas diagnosticadas y la coincidencia entre el diagnóstico clínico y el histopatológico. Método: se realizó un estudio retrospectivo de las biopsias procesadas en cinco años en el Departamento de Patología de la Facultad de Estomatología de La Habana atendiendo a edad y sexo del paciente, tipo de lesión, localización anatómica y coincidencia entre el diagnóstico clínico y el histopatológico. Resultados: se encontró que de las lesiones proliferativas, las pseudotumorales aportaron el 18,1 por ciento y las neoplasias benignas el 5,8 por ciento. En el grupo de edad de 10 a 19 años las lesiones pseudotumorales representaron el 7,78 por ciento y las neoplasias benignas el 1,78 por ciento. En el grupo de 60 años o más, las lesiones pseudotumorales aportaron el 22,67 por ciento del total de lesiones proliferativas. El sexo femenino resultó afectado por lesiones pseudotumorales en 61,3 por ciento y las neoplasias benignas afectaron al sexo masculino en un 55 por ciento. La hiperplasia fibroepitelial representó el 54,22 por ciento de las pseudotumorales, mientras que el nevus aportó el 8,67 por ciento entre las neoplasias benignas. Los diagnósticos clínicos de las lesiones pseudotumorales coincidieron en un 50,4 por ciento con los histológicos, para las neoplasias benignas la coincidencia fue del 53,2 por ciento. Conclusiones: las lesiones pseudotumorales predominaron sobre las neoplasias benignas, con un incremento marcado de ambos tipos de lesiones a partir de los 40 años. En el sexo femenino predominaron las lesiones pseudotumorales, y en el masculino predominaron las neoplasias benignas. Se encontró una baja coincidencia entre el diagnóstico clínico e histopatológico para ambos tipos de lesiones(AU)

Introduction: proliferative lesions are entities that are present in the buccal cavity. Some of them are of traumatic origin and others are neoplasias. Frequently, they are not well diagnosed in the clinical practice because of the similarity that can exist among them. Objective: to determine the characteristics of the proliferative lesions diagnosed and the coincidences between clinical and histopathological diagnosis Method: a retrospective study of the biopsies processed during five years in the Department of Oral Pathology of the Faculty of Stomatology of Havana was conducted dealing with age, sex, type of lesion, anatomic location and similarities between the clinical and histopathological diagnosis. Results: it was found that, of the proliferative lesions, the pseudotumoral ones reported the 18.1 percent and the benign neoplasias, the 5.8 percent. In the age group 10-19 years, pseudotumoral lesions represented the 7.78 percent and the benign neoplasias the 1,78 percent. In the group of 60 years or older, pseudotumoral lesions reported the 22.67 percent of the total of proliferative lesions. The female sex resulted to be affected by pseudotumoral lesions in a 61,3 percent and the benign neoplasias effected the male sex in a 55 percent. Fibroepithelial hyperplasia represented the 54.22 percent of the pseudotumoral ones, whereas the nevus reported the 8.67 percent among the benign neoplasias. The clinical diagnosis of the pseudotumoral lesions matched up a 50.4 percent with the histological; for benign neoplasias, the coincidence was 53.2 percent. Conclusions: pseudotumoural lesions predominated on benign neoplasias, with a marked increase in both types of lesions after 40 years. Pseudotumoural lesions predominated in the female sex, whereas the benign neoplasias predominated in the male sex. There was a low coincidence between the clinical and histopathological diagnosis for both types of lesions(AU)

A unique case of hereditary bilateral segmental neurofibromatosis on the face / Neurofibromatose segmentar bilateral hereditária da face: caso único

Segmental neurofibromatosis is a rare clinical finding generally with no family history and facial involvement. There are four subtypes of segmental neurofibromatosis: true segmental, localized cases with deep involvement, hereditary segmental and bilateral segmental neurofibromatosis. Here we report three patients from the same family (father, son and granddaughter) with segmental bilateral neurofibromatosis on the face. This form hasn't noticed in the literature.

A neurofibromatose segmentar é um achado clínico raro, geralmente com história familiar negativa e raro envolvimento facial. Existem quatro subtipos de neurofibromatose segmentar: segmentar verdadeira, segmentar com envolvimento visceral profundo, segmentar com história familiar e segmentar cutânea bilateral. Aqui nós reportamos três pacientes de uma mesma família (pai, filho e neta) com neurofibromatose segmentar bilateral na face. Esta forma ainda não foi relatada na literatura.

Basal cell carcinoma appearing in a facial nevus sebaceous of Jadassohn: dermoscopic features / Carcinoma basocelular aparecendo em um nevo sebáceo de Jadassohn: características dermatoscópicas

The nevus sebaceous of Jadassohn usually affects the face or scalp. It tends to evolve in three stages, and the final stage is characterized by the appearance of tumours. We present the case of a facial nevus sebaceous of Jadasshon in which a basal cell carcinoma developed. We also explore the diagnosis of this disease, which was established through dermoscopy, and propose using this technique in the clinical follow-up of this type of hamartoma, thereby allowing the early detection of cancer development.

O nevo sebáceo de Jadassohn geralmente afeta a face ou o couro cabeludo. A sua tendência natural é evoluir em três estágios, sendo que o estágio final é caracterizado pelo aparecimento de tumores. Apresentamos o caso de um nevo sebáceo de Jadassohn na face a partir do qual um carcinoma basocelular se desenvolveu. Também abordamos o diagnóstico dessa doença, estabelecido por meio da dermatoscopia. Sugerimos a utilização dessa técnica no acompanhamento clínico desse hamartoma, permitindo assim a detecção precoce de um câncer.

Case for diagnosis / Caso para diagnóstico

Atypical fibroxanthoma is a rare cutaneous tumor found mainly in elderly people on sun-exposed areas of the body. Histologically, atypical fibroxanthoma is considered a malignant fibrous histiocytoma with bizarre neoplastic cells, marked pleomorphism, hyperchromatic nuclei and abundant mitoses. It must be differentiated from other skin tumors, usually by immunohistochemistry, since its diagnosis is made by exclusion.

O fibroxantoma atípico é um tipo de neoplasia cutânea maligna rara, encontrado principalmente em idosos, em áreas fotoexpostas. Na histologia, o fibroxantoma atípico é uma neoplasia fibro-histiocítica dérmica, de células fusiformes e epitelioides, algumas vezes bizarras, com acentuado pleomorfismo, apresentando núcleos hipercromáticos e mitoses abundantes. Deve ser diferenciado de outros tumores de pele, principalmente através da imunoistoquímica, já que seu diagnóstico é de exclusão.

Double rhomboidal flap for reconstruction of large surgical defect of the labial commissure / Retalho romboidal duplo para reconstrução de defeito cirúrgico da comissura labial

Closure of perioral surgical defects involving the oral commissures is highly challenging. We describe a 69-year-old male patient with a large basal cell carcinoma of the right perioral region, extending to the right oral commissure. This lesion was radically excised, and the resulting surgical defect was closed using a homolateral double opposing rhomboidal flap. The final result was esthetically very satisfactory, with total preservation of lip function. Double opposing rhomboidal flaps are viable surgical options for the reconstruction of surgical defects involving the perioral area and oral commissures. In this relatively simple procedure, donor skin is obtained from the nearby cheek and mandibular areas, under low risk of surgical complications, preserving lip function without distortion of the labial anatomy.

A abordagem de defeitos cirúrgicos da região perioral, em particular das comissuras labiais, é um desafio importante na prática cirúrgica dermatológica. Apresentamos o caso de um doente do sexo masculino, de 69 anos, com extenso carcinoma basocelular ulcerado da região perioral direita, com envolvimento da comissura labial. Foi realizada excisão radical da lesão e reconstrução do defeito cirúrgico com retalho romboidal duplo das regiões geniana e mandibular homolaterais. O resultado cosmético final foi satisfatório, com preservação da funcionalidade das estruturas labiais. O retalho romboidal duplo é uma alternativa viável para a reconstrução de defeitos cirúrgicos da comissura labial, de execução relativamente simples, com baixo risco de complicações, que oferece óptimos resultados funcionais e cosméticos, sem distorção da anatomia labial.