Adenoma pleomorfo originado en pared lateral nasal: reporte de un caso clínico / Pleomorphic adenoma of lateral nasal wall: a case report

RESUMEN El adenoma pleomorfo constituye la neoplasia benigna más frecuente de las glándulas salivales mayores, y puede también presentarse en otros sitios con mucha menor frecuencia como orofaringe, hipofaringe y nasofaringe. El adenoma pleomorfo intranasal es muy infrecuente y los casos descritos en la literatura local se refieren a tumores septales. Se presenta un caso clínico de una paciente que consulta por obstrucción nasal unilateral a derecha asociado a, epistaxis y epífora ipsilateral con estudio imagenológico y biopsia que sugiere adenoma pleomorfo de la pared lateral nasal. Se realiza revisión bibliográfica al respecto.

ABSTRACT The pleomorphic adenoma is the most frequent benign neoplasm of the major salivary glands. It can also present itself in other places with much less frequency such as oropharynx, hypopharynx and nasopharynx. The intranasal pleomorphic adenoma is very unusual and the cases described in the local literature address septal tumors. A clinical case is presented of a patient who consulted for unilateral right nasal obstruction associated with epistaxis and ipsilateral epiphora with imaging study and biopsy suggesting pleomorphic adenoma of the lateral nasal wall. A bibliographic review is made in this regard.

[Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report]. / Linfoma T/NK nasal en granulomatosis con poliangeítis. Reporte de un caso.

BACKGROUND: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. CLINICAL CASE: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection. It was performed an oropharynx lesion biopsy and the diagnosis was extranodal NK-T-cell lymphoma, nasal type, and positive for CD56 and granzyme. CONCLUSION: Extranodal NK-T-cell lymphoma, nasal type is a rare entity, of poor prognosis, that should be considered as a diagnosis in patients with GP unresponsive to steroid. That is the reason why biopsy of the lesion and immunohistochemistry are required.

Introducción: la granulomatosis con poliangeítis (GP) es una vasculitis sistémica necrosante con afección multiorgánica que afecta principalmente el tracto respiratorio y los riñones. La fiebre en estos pacientes se considera indicador de actividad, pero si se presenta de forma aislada, deben descartarse otras causas. Caso clínico: paciente de sexo masculino que ingresó por fiebre de origen desconocido y pérdida de peso; se le diagnosticó granulomatosis con poliangeítis por antecedente de glomerulonefritis pauciinmune, nódulos pulmonares fijos y sinusitis crónica de cuatro años de evolución; sin embargo, la fiebre persistió a pesar del tratamiento y en ausencia de infección. Se realizó biopsia de úlcera faríngea que reportó linfoma de células T/NK de tipo nasal ulcerado positivo para CD56 y granzima. Conclusión: el linfoma T/NK nasal es una rara entidad, de mal pronóstico, que debe considerarse en pacientes con GP que no responden a esteroide, por lo que requieren biopsia de la lesión e inmunohistoquímica.

Tumor mesenquimatoso fosfatúrico de fosa nasal con compromiso intracraneano: reporte de un caso y revisión de la literatura / Nasal fossa phosphaturic mesenchymal tumor with intracraneal compromise: case report and review of the literature

El tumor mesenquimatoso fosfatúrico (TMF) es una enfermedad extremadamente rara. Según evidencia reciente es causado por la sobreexpresión del factor de crecimiento fibroblástico 23 (FGF23), el cual genera hipofosfemia y osteomalacia. A continuación presentamos el caso de un paciente de 42 años con un tumor mesenquimatoso fosfatúrico de fosa nasal izquierda con extenso compromiso intracraneano. Cabe destacar que hasta la fecha hay 142 casos reportados de TMF en la literatura de los cuales solo 11 se ubican en fosa nasaly cavidades sinusales, y sólo dos de ellos ubicados en fosa nasal¹. El paciente tuvo una exitosa resolución quirúrgica con la consecuente normalización de parámetros analíticos (incluido el FGF23), mejoría sintomática y ausenia de recidiva hasta la fecha.

The phosphaturic mesenchymal tumor (PMT) is an extremely rare disease. According to recent evidence is caused by overexpression of fibroblast growth factor 23 (FGF23) which generates hypophosphatemia and osteomalacia. We report the case of a 42 year old patient with a left nasal fossa phosphaturic mesenchymal tumor with intracranial involvement. Should be noted that to date there are 142 reported cases of PMT in the literature of which only 11 are located in nasal fossa and sinus cavities, two of them located in nasal fossa¹. The patient had a successful surgical resolution with consequent normalization of analytical parameters (including FGF23), absence of symptoms and no recurrence to date.

Oncogenic osteomalacia: loss of hypophosphatemia might be the key to avoid misdiagnosis / Osteomalacia oncogênica: a perda de fosfatemia pode ser a chave para evitar o diagnóstico incorreto

Diagnosing oncogenic osteomalacia is still a challenge. The disorder is characterized by osteomalacia caused by renal phosphate wasting and low serum concentration of 1,25-dihydroxyvitamin D3 occurring in the presence of a tumor that produces high levels of fibroblast growth factor 23. However, it is possible that the disease is much more misdiagnosed than rare. We present the case of a 42-year-old man with a long-term history of undiagnosed progressive muscle weakness. His laboratory results mainly showed low serum phosphate. Surgical removal of a nasal hemangiopericytoma that had been diagnosed five years earlier, brought him to a symptom-free condition. Even though knowing the underlying etiology would explain his osteomalacia, the patient sought medical help from countless physicians for five consecutive years, and only after adequate treatment a rewarding outcome was achieved. Arq Bras Endocrinol Metab. 2012;56(8):570-3.

A osteomalacia oncogênica é um diagnóstico clínico desafiador, caracterizado pela perda renal de fosfato e baixos níveis de 1,25-di-hidroxivitamina D3, ocorrendo na presença de um tumor produtor de altos níveis de fator de crescimento de fibroblasto 23. No entanto, é possível que se trate muito mais de uma falha de diagnóstico clínico do que propriamente uma doença rara. Os autores relatam o caso de um homem de 42 anos com histórico de fraqueza muscular progressiva por cinco anos e restrição à cadeira de rodas, sem diagnóstico. Seus exames laboratoriais evidenciavam baixos níveis de fósforo. A remoção cirúrgica de um hemangiopericitoma detectado previamente em cavidade nasal levou à resolução completa dos sintomas. Os autores enfatizam que, mesmo com a etiologia já evidenciada, o paciente consultou diversos clínicos no decorrer dos cinco anos até que fossem instituídos o diagnóstico e o tratamento adequados. Arq Bras Endocrinol Metab. 2012;56(8):570-3.

Oncogenic osteomalacia: loss of hypophosphatemia might be the key to avoid misdiagnosis.

Diagnosing oncogenic osteomalacia is still a challenge. The disorder is characterized by osteomalacia caused by renal phosphate wasting and low serum concentration of 1,25-dihydroxyvitamin D3 occurring in the presence of a tumor that produces high levels of fibroblast growth factor 23. However, it is possible that the disease is much more misdiagnosed than rare. We present the case of a 42-year-old man with a long-term history of undiagnosed progressive muscle weakness. His laboratory results mainly showed low serum phosphate. Surgical removal of a nasal hemangiopericytoma that had been diagnosed five years earlier, brought him to a symptom-free condition. Even though knowing the underlying etiology would explain his osteomalacia, the patient sought medical help from countless physicians for five consecutive years, and only after adequate treatment a rewarding outcome was achieved.

Esthesioneuroblastoma presenting with complete external ophthalmoplegia.

An 85-year-old male presented with unilateral third, fourth, and sixth cranial nerve palsies. Neuroimaging revealed a mass involving the medial wall of the left cavernous sinus, without orbital involvement. Biopsy of the mass provided a pathologic diagnosis of esthesioneuroblastoma.

Nasal-type NK/T-cell lymphoma presenting as hemophagocytic syndrome in an 11-year-old Mexican boy.

Recently established by the World Health Organization classification, "nasal" and "nasal-type" NK/T-cell lymphoma arise from natural killer (NK) cells. They have distinct clinicopathologic features, specific genotype and phenotype, and a high association with Epstein-Barr virus infection. Nasal-type NK/T-cell lymphoma arise from extranasal sites including skin, soft tissue, gastrointestinal tract, liver, spleen, testes, lung, and central nervous system. Most cases are reported from Asia and South America in adults. There are very few cases reported in the pediatric age group. We report an 11-year-old child with extranodal nasal-type NK/T-cell lymphoma presenting as hemophagocytic syndrome and multiorgan system failure with fatal outcome.

Papiloma invertido nasossinusal: experiência em 30 casos / Inverted sinonasal Papilloma: 30 cases experience

O papiloma invertido nasossinusal (PINS) representa de 0,5% a 4% de todos os tumores nasossinusais, e apresenta controvérsias quanto à sua etiologia, diagnóstico e tratamento.Objetivo: Mostrar nossa experiência em 30 casos de PINS através da avaliação diagnostica, conduta, recorrência e associação com malignização, comparando nossos resultados com os da literatura. Materiais e métodos: Foi realizado um estudo retrospectivo de 30 casos de PINS tratados no período de 1985 a 1998. Resultados: As queixas mais comuns foram obstrução nasal unilateral, rinorréia mucopurulenta, epistaxe e cefaléia. Os locais de acometimento mais comuns foram a fossa nasal, seio maxilar e seio etmoidal. Conclusão: O papiloma invertido nasossinusal é infreqüente, está associado à malignidade em 10% dos casos, e a via utilizada para ressecção deve ser a sublabial com degloving médio facial, com maxilectomia medial, onde não houve recorrência

Inverted sinonasal papilloma (ISP) represents 0,5% a 4% of all sinonasal tumors and we still have controversies about your etiology, diagnoses, malignancy and surgical management. Objective: We demonstrate our experience in 30 cases of ISP by diagnostic evaluation, management, recurrence and association with squamous cell carcinome and compare our results with the literature. Material and Method: We did a retrospective study of 30 cases of ISP between 1985 and 1998. Results: The most common symtoms are unilateral nasal obstruction, purulent rhinorrhea, epistaxis and headache. The usual sites include nasal cavity, maxillary and ethmoidal sinus. Conclusions: ISP is rare, associated with malignancy in 10% of cases and the usual approach to resection must be medial maxillectomy with medio-facial degloving, which provides no recurrence in our statistics.

Sinonasal lymphoma.

The clinical course of 49 patients with the diagnosis of non-Hodgkin's lymphoma were studied in the Department of Otolaryngology of the Hospital General de Mexico between December 1986 and March 1997. The most frequent clinical symptoms in these patients were: nasal obstruction, rhinorrhea, fever, weight loss, cervical lymph adenopathy, rhinopharyngeal tumor, ulceration on the palate and periorbital cellulitis. In 73% of the patients in this series the primary presentation was nasal. Sixty-six percent of the patients were classified as intermediate grade lymphoma according to the New Working Formulation, and 33% were in a I B state according to the Ann Arbor predominant immunotype was B cell in 63% of the cases. An extensive review of the literature is also presented.

V-Y flap reconstruction for nostril defects.

Local flaps are the procedure of choice in reconstruction of skin defects of the face if the donor defect is minimal. The sliding V-Y subcutaneous island flap is gaining in popularity but has been primarily used in dorsal defects of the nose. Patients demonstrating its application in nostril defects are presented. Indications for the use of this flap continue to expand.

Reparaçäo das deformidades cutâneas secundárias do nariz / Repair of secondary cutaneous deformities of the nose

Empregando princípio matemático, propöe-se a elaboraçäo de retalhos que se transpöem e justapöem de maneira harmônica e proporcional. Utilizando estes retalhos na reparaçäo das deformidades cutâneas secundárias às ressecçöes de basoepiteliomas da ponta nasal, obtêm-se resultados que tornam o método recomendável, pois há preservaçäo do contorno da regiäo e das cores e texturas locais. Ao se comparar com os métodos descritos na literatura atinente, reforça-se a propositura, pois a elaboraçäo dos retalhos é facilmente reprodutível, dado o esquema em que se baseia. Outrossim, embora a pele da ponta nasal seja pouco elástica, os retalhos säo suturados entre si sem tensäo, com adequado fechamento das áreas doadoras. Postula-se o emprego desta técnica para reparaçäo de uma regiäo rica de pormenores, sendo que as cicatrizes remanescentes ficam bem camufladas

Nasal tumour in a sheep in Trinidad, West Indies

Medical treatment of a pregnant 3 to 4-year old Barbados Black Belly ewe with a swelling of the left face and from which there was a bloody nasal discharge was unsuccessful. Further clinical examination of the animal led to the suspicion of a malignant neoplasm. The animal was euthanised and a detailed post mortem examination was performed. Gross and histological examination revealed an adenocarcinoma of the nasal cavity with metastasis to the lungs. Tumours in sheep generally, and adenocarcinomas of the nasal cavity in particular, are of rare occurence (AU)