[Cerebrospinal fluid rhinorrhea secondary to ethmoidal carcinoma: case report]. / Rinorrea de líquido cefalorraquídeo secundaria a carcinoma etmoidal: reporte de caso.

BACKGROUND: The first report of cerebrospinal fluid rhinorrhea (CSFR) was described in 1679. In 1826 it was reported that one of the possible causes of CSFR was a fistula between the subarachnoid space and the nasal cavity. In 1903, chemical analysis of the fluid was proposed as a diagnostic criterion. In Mexico there has been 32 case reports. CASE REPORT: Forty-nine years old female with a history of nasal polyposis, profuse rhinorrhea and cephalea who attends the allergy department with the suspicion of allergic rhinitis. After anamnesis and physical evaluation, CSFR was suspected. Chemical analysis of the fluid, head CT and biopsy of nasal polyp were performed. An etmoidal fistula associated with carcinoma was confirmed. CONCLUSIONS: Spontaneous fistulas are rare but can erosionate the bone and adjacent tissues. Diagnosis is based on the clinical findings, patient's history and complementary studies such as beta-2-transferrin determination in nasal fluid.

ANTECEDENTES: En 1679 se describió el primer caso de rinorrea de líquido cefalorraquídeo. En 1826 se reportó como causa una fistula entre el espacio subaracnoideo y la cavidad nasal. Para 1903 se propuso el análisis químico como criterio diagnóstico. En México sólo se han reportado 32 casos de rinorrea de líquido cefalorraquídeo. REPORTE DE CASO: Paciente femenina de 49 años, con antecedente de poliposis nasal, rinorrea abundante y cefalea, quien acudió a consulta para descartar rinitis alérgica. Luego de la anamnesis y la exploración física se sospechó de fuga de líquido cefalorraquídeo secundaria a fístula nasal. Con la histoquímica de moco, tomografía de cráneo y biopsia del pólipo nasal se estableció el diagnóstico de fístula etmoidal secundaria a carcinoma. CONCLUSIÓN: La fístulas espontáneas son excepcionales, pueden erosionar el hueso y los tejidos adyacentes. El diagnóstico se establece con la historia clínica y los antecedentes médicos, además de estudios complementarios y la determinación de Beta-2-transferrina en moco.

FGF23-secreting sinonasal tumour presenting with acute subdural haemorrhage and tumour-induced osteomalacia.

A female in her 50s developed a headache, collapsed and was noted to have an acute atraumatic subdural haemorrhage (SDH) requiring surgical evacuation and intracranial pressure-directed therapy. Her background included recurrent epistaxis, severe generalised bone pain and multiple insufficiency fractures and an undifferentiated autoimmune connective tissue disease. Chronic hypophosphataemia, elevated alkaline phosphatase and raised fibroblast growth factor 23 (FGF23) were also noted. An MRI head and subsequent 68Ga CT/positron emission tomography scan demonstrated an intensely avid tumour in the right ethmoid sinus, extending intracranially. Phosphate was aggressively replaced, and alfacalcidol was initiated to circumvent the effects of FGF23 on her kidneys and bone minerals. The tumour was biopsied and then definitively resected via combined endonasal and craniotomy approaches, resulting in good clinical improvement. FGF23 titre and serum phosphate both normalised leaving the diagnosis of a phosphaturic mesenchymal tumour-secreting FGF23, leading to tumour-induced osteomalacia.

Hipo, un desafío diagnóstico / Hiccup, a diagnostic challenge

El hipo es una contracción espasmódica involuntaria del diafragma que desencadena una inspiración súbita y cierre abrupto de la glotis originando un sonido característico. Según su duración se clasifica en ataque de hipo, hipo persistente o hipo intratable o rebelde. Presentamos el caso de un varón de 75 años de edad con hipo rebelde que le impedía conciliar el sueño, diurno y nocturno, asociado a eructos, de tres años de evolución, refractario al tratamiento con clorpromazina. Luego de una extensa evaluación se llegó al diagnóstico de tumor en seno etmoidal derecho. Se efectuó la resección del tumor y posterior radioterapia local con desaparición del hipo. Consideramos ésta una causa rara de hipo rebelde que generó un desafío diagnóstico y terapéutico.

Hiccup is a spasmodic involuntary contraction of the diaphragm which triggers a sudden inspiration and an abrupt closure of the glottis with a characteristic sound. Regarding its duration, it is classified as hiccup attack, persistent hiccup or rebellious or intractable hiccup. We present the case of a 75 old male with rebellious hiccup which prevented him to fall asleep by day or by night, associated with belches lasting for three years, refractory to chlorpromazine. An extensive evaluation led to the diagnosis of right ethmoid sinus tumor. The tumor resection was performed and after subsequent local radiotherapy the hiccup disappeared. We consider this a rare cause of rebellious hiccup that generated a diagnostic and therapeutic challenge.

Osteomalacia inducida por tumor: hemangiopericitoma rinosinusal / Tumor-induced osteomalacia: rhinosinusal hemangiopericytoma

La osteomalacia inducida por tumor es una rara enfermedad del metabolismo óseo caracterizada por el aumento en la excreción de fosfato a nivel renal seguido de hipofosfatemia. Es causada por agentes fosfatúricos producidos por determinados tumores. La resección total del tumor resulta en la completa reversión de las anormalidades bioquímicas, la desaparición de las manifestaciones clínicas y los hallazgos en los estudios por imágenes. Presentamos el caso de un varón de 61 años con cuadro clínico y laboratorio compatibles con osteomalacia oncogénica inducida por tumor mesenquimático de localización rinosinusal. En nuestro caso el diagnóstico histológico correspondió a una neoplasia de tipo vascular: hemangiopericitoma.

Tumor-induced osteomalacia is a rare disease of bone metabolism. The characteristic of this disease is an increase in phosphate excretion followed by hypophosphatemia, due to phosphaturic agents produced by different types of tumors. Tumor resection results in complete resolution of clinical, biochemical and radiological abnormalities. We present the case of a 61 year old man with signs, symptoms and laboratory findings consistent with oncogenic osteomalacia due to a rhino-sinusal mesenchymal tumor. The histological diagnosis showed a vascular neoplasm: hemangiopericytoma.

Pseudo-tumor pituitario e hipopituitarismo secundario a un papiloma invertido del seno esfenoidal / Pseudo-pituitary tumor and hypopituitarism secondary to a sphenoid sinus inverted papilloma

El papiloma invertido (PI) es un tumor epitelial benigno, poco frecuente, que se origina mayormente de la pared nasal lateral. A pesar de ser benigno, constituye una lesión altamente invasiva de tejidos vecinos y puede sufrir una transformación maligna. El PI primario del seno esfenoidal con extensión intracraneana e invasión dural, aun sin evidencia histológica de malignidad, ha sido excepcionalmente descrito. Describimos el caso de una mujer de 59 años de edad que fue evaluada por cefaleas intensas de 5 años de evolución y anormalidades del campo visual. Una resonancia magnética nuclear (RMN) mostró una masa selar heterogénea de 1.4 por 2 cm con extensión supraselar y al seno esfenoidal, con erosión del piso selar y compresión del quiasma óptico. Recibió 16 mg/día de prednisona durante aproximadamente 3 meses con una regresión casi total de la masa en la RMN. En la evaluación hormonal se halló insuficiencia gonadal, tiroidea y adrenal central. En una nueva RMN se observó crecimiento del tumor con compromiso total del seno esfenoidal. Una biopsia endoscópica confirmó el diagnóstico de PI. Se realizó una cirugía sinusal transnasal endoscópica con una resección completa evidenciada en una RMN un año más tarde.

nverted papilloma (IP) is a benign uncommon epithelial tumor, arising mostly from the lateral nasal wall. Though benign, this lesion is highly invasive into surrounding tissues and malignant transformation may occur. Primary IP of the sphenoid sinus and intracranial extension with dural invasion, even without histological evidence of malignancy, has only rarely been described. Hypopituitarism as a complication of this lesion has never been reported. We describe the case of a 59-year-old woman who was evaluated because of a 5-year-history of severe headaches and abnormalities in the visual field. Magnetic resonance imaging (MRI) showed a 1.4 per 2.0 cm heterogeneous sellar lesion with suprasellar and sphenoid sinus extension, eroding the sellar floor with optic chiasm compression. Otolaryngologists gave her 16 mg/day of prednisone during approximately 3 months with a near total regression of the mass on MRI. The endocrine biochemical evaluation showed pituitary gonadal, thyroid and adrenal insufficiency. A new MRI showed growth of the tumor with obliteration of the sphenoid sinus. An endoscopic sinus biopsy revealed an IP, so a transnasal endoscopic sinus surgery was performed with complete resection evidenced by MRI a year later.

Non-Hodgkin's lymphoma associated with the acquired immunodeficiency syndrome

Patients with the Acquired Immunodeficiency Syndrome (AIDS) have an increased risk of developing Non-Hodgkin's Lymphomas (NHL). It is estimated that 8-27 of newly diagnosed NHL are associated with AIDS. The majority of these lymphomas are of intermediate or high grade histology. We retrospectively analyzed the clinical manifestations and response to different chemotherapy regimens in patients with AIDS NHL in the San Juan City and San Juan Veterans Administration Hospitals from 1990-1993. Eleven patients (10 M/1F) with an average age of 46 (range 31-68) were analyzed. 64 (7/11) of patients had a prior diagnosis of AIDS before the diagnosis of NHL. Pathology was diffuse large cell in 6, Burkitt's in 4 and immunoblastic in 1.73 of patients presented with Stage III/IV. Sites included gastrointestinal tract (2), skin/soft tissue (2), paranasal sinus (1), kidneys (1). The most common treatment regimen was the modified m-BACOD (64). Complete response (CR) occurred in 46 (5/11) and partial response (PR) in 27 (3/11). 36 of patients developed opportunistic or bacterial infections during treatment. Median survival was 8 months. Two patients are long-time survivors at 24+, 29+ without evidence of NHL at present. This group of patients demonstrate the usual characteristics of NHL in AIDS patients and corroborate that conventional chemotherapy programs are only moderately effective in these patients. It is clear from this data and from other reports that further work will be required to ascertain optimal therapy for the patient with AIDS-related Non-Hodgkin's Lymphoma

Pneumatocele frontal secundário a osteoma do seio frontal / Frontal pneumatocele secundary to frontal sinus osteoma

Apresenta-se um caso de Pneumatocele intracraniano secundário a complicaçäo de osteoma do seio frontal direita. Säo apresentados: história clínica, quadro neurológico, achados neuro-radiológicos, técnica cirúrgica e resultado pós-operatório e análise da literatura pertinente ao assunto