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.

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.

Nasolacrimal Duct Obstruction Caused by Oncocytic Carcinoma

Here, we report an extremely rare case of acquired nasolacrimal duct obstruction caused by oncocytic carcinoma. A 64-year-old man presented to the hospital complaining of epiphora and left-side nasal obstruction. Ophthalmic and otolaryngology examination revealed a left lacrimal duct obstruction caused by a mass in the left nasal cavity and lacrimal drainage system. The mass was removed and confirmed as an oncocytic carcinoma. Nine months after surgery, without adjuvant radiotherapy, a left orbital mass was observed and the patient underwent reoperation. The mass proved to be recurrent oncocytic carcinoma. The patient underwent adjuvant radiotherapy to eradicate any residual tumor and the patient remains tumor-free one year post-radiotherapy.

Nasolacrimal Duct Obstruction Caused by Oncocytic Carcinoma

Here, we report an extremely rare case of acquired nasolacrimal duct obstruction caused by oncocytic carcinoma. A 64-year-old man presented to the hospital complaining of epiphora and left-side nasal obstruction. Ophthalmic and otolaryngology examination revealed a left lacrimal duct obstruction caused by a mass in the left nasal cavity and lacrimal drainage system. The mass was removed and confirmed as an oncocytic carcinoma. Nine months after surgery, without adjuvant radiotherapy, a left orbital mass was observed and the patient underwent reoperation. The mass proved to be recurrent oncocytic carcinoma. The patient underwent adjuvant radiotherapy to eradicate any residual tumor and the patient remains tumor-free one year post-radiotherapy.

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.

Mucosal malignant melanoma of the nasal cavity.

Mucosal malignant melanoma (MMM) of the nose is extremely rare. We report a case of MMM of the nasal cavity in a 60-year-old male patient presenting with a polypoidal mass in the right nasal cavity. It was increasing gradually and obstructing breathing. A biopsy of the lesion was done with a clinical suspicion of inverted papilloma/carcinoma. Microscopy revealed features suggestive of malignant melanoma with minimal melanin pigmentation. Subsequently wide local excision was done. Diagnosis of malignant melanoma was facilitated by histochemistry and immunohistochemistry.

Recurrent epistaxis from an intranasal glomus tumor: the 22nd case report.

The case of a 66-year-old Thai woman who was treated at King Chulalongkorn Memorial Hospital due to recurrent epistaxis for two years is presented. Her physical examination revealed a 0.7-cm tumor mass on the right nasal septum. A biopsy was subsequently done and the tissue was sent for pathological examination. The lesion was then diagnosed as "glomus tumor of the nasal septum". Histologically, the tumor was rather well-circumscribed and located in the submucosal region. The neoplastic cells were uniform, round to ovoid in shape and contained bland-looking, finely chromatic nuclei and moderate amount of cytoplasm with ill-defined cell borders. These cells were intervened by tortuous vascular structures. Immunohistochemically, the tumor cells were strongly reactive to smooth muscle actin and negative to cytokeratin and S-100 protein. A glomus tumor is rare in the sinonasal region. So far, there have been only 21 reported cases in the literature. Almost all cases of sinonasal glomus tumor are benign and are usually cured by complete excision.

Sinonasal and nasopharyngeal adenoidcystic carcinoma: report of four cases.

Four cases of sinonasal and nasopharyngeal adenoidcystic carcinoma that came to the RIMS Hospital, Imphal during the period of July, 2002 to March, 2003 are reported in this article. The age incidence ranged from 30 to 80 years and the mean age was 47.5 years; with male to female ratio of 1:1. The average duration from first sympton in the patient to the date of his medical consultation was 17.8 months. Nasal obstruction was the first symptom two cases; lump sensation in the throat and headache in the third and fourth cases respectively. Despite the evidence of rapid and extensive local spread there were definitely delays from the patients' end to consult a clinician. Whereas the tumour itself is not common, the usual sites, if it occurs, are palate and minor salivary glands and rarely mucous and seromucinous glands elsewhere.

Extrahepatic Metastasis of Hepatocellular Carcinoma to the Nasal Cavity Manifested as Massive Epistaxis: A Case Report / 대한간학회지

Extrahepatic metastasis of hepatocellular carcinoma (HCC) is not infrequently found during the later stage, regarding that the autopsy report described its prevalence to be up to 50%. The most frequent sites are known to be the abdominal lymph nodes, lung and bone. However, metastasis to the nasal cavity and paranasal sinuses has been seldom reported, and to out knowledge, there is no Korean report describing extrahepatic metastasis of HCC to these sites. Recently we experienced a case of extrahepatic metastasis of HCC to the nasal cavity in a 50 year-old man with massive epistaxis refractory to conservative treatment. He was found to have a mass of soft tissue attenuation occupying the right nasal cavity at CT, which was biopsy-proven as metastatic HCC. Epistaxis was successfully treated by transcatheter arterial embolization.

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.

Nasal angiocentric lymphoma with hemophagocytic syndrome

OBJECTIVES: Hemophagocytic syndrome (HS) is a fatal complication of nasal angiocentric lymphoma (AL) and difficult to distinguish from malignant histiocyosis. Epstein-Barr virus (EBV)-associated HS is frequently observed in lymphoma of T-cell lineage and EBV is highly associated with nasal AL. Clinicopathologic features of 10 nasal ALs with HS were reviewed to determine the clinical significance and the pathogenetic association with EBV. METHODS: Ten patients of HS were identified from a retrospective analysis of 42 nasal ALs diagnosed from 1987 to 1996. Immunohistochemical study and in situ hybridization were performed on the paraffin-embedded tumor specimens obtained from 10 patients. Serologic study of EBV-Ab was performed in 3 available patients. RESULTS: Five patients had HS as initial manifestation, 3 at the time of relapse and 2 during the clinical remission of AL. Four patients were treated by combination chemotherapy (CHOP) and others had only supportive care. The median survival of all patients with HS was 4.1 months (range 2 days-36.5 months) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1 (CD45RO) and EBV by EBER in situ hybridization. The data of serologic tests indicated the active EBV infection. CONCLUSIONS: HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in the future.

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