Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Asociación de hemangioma en zona cérvico facial y hemangioma subglótico en un neonato / Association of cervical-facial and subglottic hemangioma in neonates / Associação de hemangioma cervicofacial e subglótico em neonatos

Se presenta un neonato con hemangioma cérvico facial y posterior diagnóstico de hemangioma subglótico. Los hemangiomas en el período neonatal y los primeros meses de vida requieren una atención cuidadosa. Debido a su patrón de crecimiento y la futura aparición de nuevas lesiones, son considerados imprevisibles en esta etapa. Se encontró una fuerte asociación entre los hemangiomas difusos de localización cérvico facial y los hemangiomas sintomáticos de la vía aérea alta. El riesgo está relacionado con el grado de extensión de la afectación cutánea en un área que incluye la piel de la región mandibular, el mentón, el labio inferior y la parte anterior del cuello. Los hemangiomas infantiles requieren tratamiento cuando presentan riesgo vital y alteraciones funcionales, como compromiso de la vía aérea.

This is the case of a newborn with cervical hemangioma and a subsequent diagnosis of subglottic hemangioma. Hemangiomas in neonates and infants require careful attention. Due to their growth pattern and the potential appearance of new lesions, they are considered unpredictable at this stage. A strong link was found between diffuse cervical-facial and symptomatic upper airway hemangiomas. The risk is related to the extent of skin involvement in a given area, which might include the jaw, chin, lower lip, and front of the neck skin. Infant hemangiomas require treatment when they present life-threatening and functional alterations, such as airway compromise.

Relatamos o caso de um recém-nascido com hemangioma cervical com diagnóstico posterior de hemangioma subglótico. Hemangiomas em recém-nascidos e lactentes requerem atenção cuidadosa. Devido ao seu padrão de crescimento e ao potencial aparecimento de novas lesões, são considerados imprevisíveis nessa fase. Uma forte associação foi encontrada entre hemangiomas cervicofaciais difusos e hemangiomas sintomáticos das vias aéreas superiores. O risco está relacionado à extensão do envolvimento da pele da mandíbula, o queixo, o lábio inferior e a pele da frente do pescoço. Os hemangiomas infantis necessitam de tratamento quando apresentam alterações funcionais ou risco de vida, como comprometimento das vias aéreas.

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

Classification and reconstruction of complex defects after lateral facial tumor surgery / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the classification and reconstruction strategy of defects in lateral face region after operation of malignant tumors. Methods: Eighteen cases with the reconstruction of complicated defects after resection of tumors in the region of lateral face from January 2015 to January 2018 in Hunan Cancer Hospital were retrospectively reviewed. There were 14 males and 4 females, aged from 32 to 68 years. According to the presence or absence of bony scaffold, complicated defects were divided into two main categories: soft tissue perforating defects and soft tissue defects combined with bony scaffold defects. All soft tissue perforating defects in 5 cases were repaired with free anterolateral femoral flaps. Among 13 cases with soft tissue plus bony scaffold defects, 3 were repaired with free fibular flaps, 6 with free fibular flaps combined with free anterolateral femoral flaps, and 4 with chimeric deep circumflex iliac artery perforator flaps combined with anterolateral femoral flaps. Results: All flaps survived well. Two patients complicated with fistula in floor of mouth, but the wound healed after dressing change. Transoral feeding was resumed within 2 weeks after surgery in all patients. One year follow-up evaluation showed that 14 cases had symmetrical face and 10 cases had mouth opening more than 3 transverse fingers. After 36-50 months of follow-up, 6 patients died, with an overall 3-year survival rate of 66.7%. Conclusion: The classification of defects with or without bony stent loss is conducive to the overall repair design, the recovery of facial contour stent, the effective fill of dead space and the maintain of residual occlusal relationship. Good reconstruction results require a multi flap combination of osteocutaneous and soft tissue flaps.

Versatilidad del colgajo miofascial de temporal para la reconstrucción tras cirugía oncológica / Versatility of the temporal myofascial flap for reconstruction after oncological surgery

RESUMEN Introducción: La magnitud de las resecciones de tumores de cabeza y cuello, depende del tamaño tumoral, la adecuación de los márgenes y factores inherentes al paciente. El músculo temporal, constituye una excelente opción para reconstruir defectos de zonas aledañas a su sitio de implantación. Objetivo: Demostrar la versatilidad del colgajo miofascial de temporal para la reconstrucción tras, cirugía oncológica de cabeza y cuello, en el Hospital Provincial Universitario "Camilo Cienfuegos Gorriarán" desde enero de 2017 a diciembre de 2018. Métodos: Se realizó una revisión retrospectiva de los informes operatorios de los casos intervenidos con cirugía oncológica de cabeza y cuello, en el servicio de cirugía maxilofacial; se tomó de muestra a pacientes a los que, se les realizó reconstrucción del defecto quirúrgico, utilizándose colgajo miofascial de temporal. Resultados: Fueron sometidos a reconstrucción, 12 pacientes de forma inmediata, en un solo tiempo quirúrgico. El defecto se presentó mayormente en lesiones de piel. La edad promedio de los pacientes fue 66,2 años. Se observó una distribución equitativa en cuanto a sexo, con 6 pacientes para cada grupo. El seguimiento osciló entre 2 y 20 meses, con una media de 11,25 meses, de los cuales todos los pacientes se encontraban vivos y controlados. Conclusiones: El colgajo temporal continúa siendo una opción válida para la reconstrucción en cirugía oncológica de cabeza y cuello. En la serie revisada no ocurrió necrosis, lo que, lo hace un colgajo muy seguro, con buenos resultados estéticos y funcionales y con buena tolerancia ante los tratamientos oncoespecíficos adyuvantes(AU)

ABSTRACT Introduction: The magnitude of the resections of head and neck tumors depends on the tumor size, the adequacy of the margins and factors inherent to the patient. The temporal muscle is an excellent option for reconstructing defects in areas surrounding its implantation site. Objective: To demonstrate the versatility of the temporal myofascial flap for reconstruction after oncological head and neck surgery, at the "Camilo Cienfuegos Gorriarán" Provincial University Hospital from January 2017 to December 2018. Methods: A retrospective review of the operative reports of the cases intervened with oncological head and neck surgery was performed in the maxillofacial surgery service; A sample was taken from patients who underwent reconstruction of the surgical defect, using a temporal myofascial flap. Results: 12 patients underwent reconstruction immediately, in a single surgical time. The defect occurred mainly in skin lesions. The average age of the patients was 66.2 years. An equitable distribution in terms of sex was observed, with 6 patients for each group. Follow-up ranged from 2 to 20 months, with a mean of 11.25 months, of which all the patients were alive and controlled. Conclusions: The temporary flap continues to be a valid option for reconstruction in head and neck cancer surgery. Necrosis did not occur in the revised series, which makes it a very safe flap, with good aesthetic and functional results and with good tolerance to adjuvant oncospecific treatments(AU)

Giant frontal lipoma mimicking frontal mucocele / Lipoma frontal gigante mimetizando mucocele frontal

ABSTRACT Lipoma is a usually painless benign adipose tumor. Nevertheless, it can cause compression of adjacent structures, depending on its location. Mucoceles are benign cystic lesions in the frontal-ethmoidal region, caused by chronic obstruction of the paranasal sinus and consequent inflammatory processes. The increase of mucocele diameter caused by inflammation often results in bony degradation and reabsorption, increasing pressure on adjacent structures, including the orbit and the base of the skull, leading to possibly serious consequences. Here we report a case of lipoma mimicking a frontal mucocele, managed surgically at the Ophthalmological Hospital of Anapolis.

RESUMO O lipoma é um tumor do tecido adiposo de caráter benigno, usualmente indolor, porém que pode gerar compressão de estruturas adjacentes, dependendo da sua localização. A mucocele consiste em uma lesão cística de caráter benigno, na região fronto-etmoidal, devido à obstrução crônica dos óstios dos seios paranasais e consequente processo inflamatório. O aumento do diâmetro da mucocele por inflamação muitas vezes causa degradação e reabsorção ósseas, o que pode elevar a pressão em estruturas adjacentes como órbita e base de crânio, causando intercorrências possivelmente graves. Neste estudo relatamos um caso de lipoma simulando mucocele frontal, com propedêutica cirúrgica.

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.

PHACE syndrome: clinical manifestations, diagnostic criteria, and management

Abstract: Infantile hemangioma can be linked to other organ malformations. In 1996, PHACE syndrome was first defined as the association of large and segmental infantile hemangioma, usually on the face, head, or cervical region, with malformations of the posterior fossa of the brain, arterial anomalies of the central nervous system, coarctation of the aorta, cardiac defects, and ocular abnormalities. Over 300 cases of PHACE syndrome have been reported, and it is cconsidered one of the most common neurocutaneous vascular disorders in childhood. Knowledge of the features and locations of lesions that imply a greater risk of systemic involvement is crucial for the diagnosis and proper management of PHACE syndrome patients. This review highlights the diagnostic criteria for PHACE syndrome, the imaging workup for extracutaneous involvement, the treatment of infantile hemangioma, and the importance of a multidisciplinary approach in the management of these patients.

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.

Malignant pilomatricoma of the cheek in an infant

Malignant pilomatricoma (pilomatrical carcinoma) is a rare, locally occurring malignant tumor with a high rate of recurrence in the case of incomplete excision. This tumor has two characteristics. First, recurrences of pilomatrical carcinoma are common; second, distant metastasis is rare, but if it occurs, it is very fatal. It has characteristic features of high mitotic counts, cellular atypia, and local invasion. Although fine needle aspiration and excisional biopsy could help to confirm this tumor diagnosis, pathologic findings are critical. Pilomatricomas have some characteristic features in histological aspect, such as epithelial islands of basaloid cells and shadow cells or ghost cell. Also, various types of immunohistochemical staining are used to confirm the diagnosis. Despite the lack of clear surgical criteria, treatment is a wide local excision with histologically clear resection margins with or without adjuvant radiotherapy.

Cáncer Bucomaxilofacial en Niños III: Tumores Malignos del Tejido Blando / Oral and Maxillofacial Cancer in Children III: Malignant Soft Tissue Tumors

RESUMEN: El grupo de neoplasias malignas de tejido blando de la región de cabeza y cuello en pacientes pediátricos está representado por carcinomas, sarcomas, melanomas y tumores de diferenciación incierta. La neoplasia más prevalente en la población pediátrica es el Rabdomiosarcoma, seguido por el carcinoma de células escamosas. Los rangos de presentación son muy amplios, siendo los grupos entre 2-6 años y 15-19 años los que presentan mayor incidencia. Se ha planteado que la etiología de estas neoplasias es incierta. El tratamiento de estas neoplasias es comúnmente de enfoque multimodal, combinando un procedimiento quirúrgico con quimioterapia y radioterapia. El pronóstico y sobrevida del paciente dependerán principalmente del momento en que se realice el diagnóstico de la lesión. Un diagnóstico y tratamiento temprano favorecen las posibilidades de sobrevida y el pronóstico del paciente. Este estudio corresponde a la 3ra parte de "Cáncer bucomaxilofacial en niños". Se hará referencia a los distintos tumores malignos del tejido blando en la población pediátrica en el territorio de cabeza y cuello, abarcando sus generalidades, etiología, epidemiología, tratamiento y pronóstico.

ABSTRACT: Head and neck malignant tumors in pediatric patients comprise carcinoma, sarcoma, melanoma and tumours of uncertain differentiation. Within the pediatric population, the most prevalent neoplasm is rhabdomyosarcoma, followed by squamous cell carcinoma. There is a wide range in the presentation, and it varies significantly with age groups of 2-6 and 15-19 year-olds who present the higher incidence rates. For this reason, it has been suggested that the etiology of head and neck neoplasms remains unclear. Treating these pathologies usually involves a multimodal approach that combines surgery, radiation and chemotherapy. Prognosis and survival rates depend mainly of the stage at the time of diagnosis. Early diagnosis and treatment can improve prognosis and survival rates. In this 3rd part of "Maxillofacial Cancer in Pediatric Patients", we studied a variety of malignant tumors in head and neck soft tissue from a paediatric sample. Specifically, we aim to analyze their etiology, epidemiology, treatment and prognosis.

Tunneled island flaps in facial defects reconstruction

Abstract The reconstruction of facial surgical defects is usually challenging for the dermatologic surgeon. Three different cases of facial defects in which tunneled island flaps were used are reported. In 2 cases, wide defects involving the nasal dorsum and ala were repaired using a nasolabial island flap tunneled through the lateral side of the nose. A tunneled island glabellar flap was used for medial canthus reconstruction in the third case. Despite complex pedicle dissection and frequent trapdoor deformation, tunneled island flaps allow reconstruction of wide defects in a single-staged procedure, camouflaging the scar of the donor area in boundaries of cosmetic units and preserving the facial central symmetry.

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.

Chondroid Syringoma on Face

Chondroid syringoma is a rare mixed tumor of the skin which is composed of both mesenchymal and epithelial cells. Its incidence at less than 0.1% and is frequently located on the head and neck. Chondroid syringoma is easily confused with epidermal cysts. Since malignant forms of chondroid syringoma have been reported, accurate and timely diagnosis is important for proper management. We report clinical and histological features of chondroid syringoma in 5 patients treated at our institution. In most of the cases, chondroid syringoma presented as a round, firm, nodular or cystic lesion that had well marginated heterogeneity in sonography. Clinically, all of the lesions were removed by simple excision. Microscopically, all five tumors were well circumscribed and consisted of epithelial, myoepithelial, and stromal components. The epithelial component formed tubules lined by one or more rows of eosinophilic epithelial cells. The outer layer of tubules appeared to be flattened myoepithelial cells. The stroma is myxoid and contained spindle shaped myoepithelial cells. We expect that the clinical, sonographic, and histological data from our report may help clinicians who are confronted with various kinds of analogous facial lesions to decide the most proper management for their patients.

Chondroid Syringoma on Face

Chondroid syringoma is a rare mixed tumor of the skin which is composed of both mesenchymal and epithelial cells. Its incidence at less than 0.1% and is frequently located on the head and neck. Chondroid syringoma is easily confused with epidermal cysts. Since malignant forms of chondroid syringoma have been reported, accurate and timely diagnosis is important for proper management. We report clinical and histological features of chondroid syringoma in 5 patients treated at our institution. In most of the cases, chondroid syringoma presented as a round, firm, nodular or cystic lesion that had well marginated heterogeneity in sonography. Clinically, all of the lesions were removed by simple excision. Microscopically, all five tumors were well circumscribed and consisted of epithelial, myoepithelial, and stromal components. The epithelial component formed tubules lined by one or more rows of eosinophilic epithelial cells. The outer layer of tubules appeared to be flattened myoepithelial cells. The stroma is myxoid and contained spindle shaped myoepithelial cells. We expect that the clinical, sonographic, and histological data from our report may help clinicians who are confronted with various kinds of analogous facial lesions to decide the most proper management for their patients.