RESUMO
BACKGROUND: Isolated limb perfusion (ILP) is an established and effective treatment for advanced melanoma and soft tissue sarcomas of the extremities with a high overall response rate. The aim of this study was to describe our experience of ILP for more rare types of tumours. METHODS: Patients with Merkel cell carcinoma (MCC) (n = 4), squamous cell carcinoma (SCC) (n = 2), B-cell lymphoma (n = 1), desmoid tumours (n = 3), pigmented villonodular synovitis (PVNS) (n = 1) and giant cell tumour (n = 1) were treated with ILP and analysed retrospectively. RESULTS: The four patients with in-transit MCC had three complete responses (CR) and one partial response (PR); the two patients with SCC had one CR and one stable disease (SD); the patients with desmoid tumours had two PR and one SD. A CR was also observed for the patient with a giant cell tumour, but the patient with PVNS had a SD. The patient with cutaneous metastases of B-cell lymphoma showed a CR, however with rapid systemic progression. Local toxicity according to Wieberdink was grade II in 10 patients (83%) and grade III in two patients (17%). CONCLUSIONS: These results show that ILP can be used as a treatment option also for more rare disease entities when other treatments have failed.
Assuntos
Quimioterapia do Câncer por Perfusão Regional , Hipertermia Induzida , Neoplasias Abdominais/tratamento farmacológico , Neoplasias Abdominais/terapia , Polipose Adenomatosa do Colo/tratamento farmacológico , Polipose Adenomatosa do Colo/terapia , Antineoplásicos Alquilantes/uso terapêutico , Carcinoma de Célula de Merkel/tratamento farmacológico , Carcinoma de Célula de Merkel/terapia , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/terapia , Extremidades , Fibromatose Agressiva/tratamento farmacológico , Fibromatose Agressiva/terapia , Tumores de Células Gigantes/tratamento farmacológico , Tumores de Células Gigantes/terapia , Humanos , Linfoma de Células B/tratamento farmacológico , Linfoma de Células B/terapia , Melfalan/uso terapêutico , Perfusão , Doenças Raras/tratamento farmacológico , Doenças Raras/terapia , Sinovite Pigmentada Vilonodular/tratamento farmacológico , Sinovite Pigmentada Vilonodular/terapia , Fator de Necrose Tumoral alfa/uso terapêuticoRESUMO
BACKGROUND: Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by osteomalacia, which occurs as a result of excess renal phosphate excretion caused by fibroblast growth factor-23 secreted by mesenchymal tumors. This entity is rare in head and neck cancers. We report a rare case of oncogenic osteomalacia in a patient with an anterior skull base giant cell tumor. METHODS AND RESULTS: A 34-year-old woman presented with a 5-year history of progressive weakness in both lower limbs and the trunk. Hypophosphatemia and hypocalcemia had been noted by a local physician, but her symptoms persisted despite receiving calcium and vitamin D supplements. A recent onset of epistaxis and nasal blockage led to referral to the head and neck services. Nasal endoscopy revealed a left nasal cavity mass. Further evaluation with imaging studies revealed a mass in the nasal cavity with intracranial extension. Biopsy of the lesion suggested a neurogenic tumor. A putative diagnosis of anterior skull base neurogenic tumor with paraneoplastic hypophosphatemia was made. After the biochemical parameters were corrected, the patient underwent craniofacial resection. The final histopathologic study suggested the lesion as a "giant cell tumor." During the postoperative period the patient's biochemical and clinical symptoms improved dramatically, allowing her to regain normal mobility. CONCLUSIONS: Clinicians and pathologists must be aware of the clinical symptoms, laboratory abnormalities, and pathologic features of oncogenic osteomalacia, which may be caused by tumors in the head and neck and thus make an exhaustive effort to diagnose the same.
Assuntos
Tumores de Células Gigantes/complicações , Cavidade Nasal , Neoplasias de Tecido Conjuntivo/diagnóstico , Neoplasias de Tecido Conjuntivo/etiologia , Neoplasias Nasais/complicações , Adulto , Feminino , Tumores de Células Gigantes/diagnóstico , Tumores de Células Gigantes/terapia , Humanos , Neoplasias de Tecido Conjuntivo/terapia , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/terapia , Osteomalacia , Síndromes ParaneoplásicasRESUMO
The paper presents results of treatment of 16 patients operated on because of giant cell tumor. Frequent local recurrence after curettage makes the authors to suggest more common use of the thermal or chemical destruction of the neoplastic cells to achieve oncologic sterility.