Radiotherapy of granulomatosis with polyangiitis occurring in the eyelid: A case report and literature review.

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a chronic systemic vasculitis characterized by necrotizing granulomatous vasculitis. The disease mainly affects the middle and small blood vessels and mainly occurs in the upper respiratory tract (nose and paranasal sinuses), lower respiratory tract (lungs), and kidneys. Disease occurrence in the eyelid area is relatively rare. The standard GPA treatment is combination therapy with adrenocortical hormone and immunosuppressants. Radiotherapy as a treatment option for GPA has not been widely investigated. PATIENT CONCERNS: A 29-year-old man presented with a 1.0 × 1.0 cm mass without exophthalmos and decreased vision in the left lower eyelid. Computed tomography revealed a mass-like high-density shadow below the left eye with a computed tomography value of 80-108 U. DIAGNOSIS: The laboratory investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies (titer = 1:40). Biopsy of the lower left eyelid mass revealed necrosis and granulomatous reaction with a large number of inflammatory cell infiltration. After consultation with the pathology department, the diagnosis was determined as left lower eyelid GPA. INTERVENTIONS: The patient received 9MeV electron beam radiation therapy in the area of the left lower eyelid lesion. OUTCOMES: The lesion in the patient was significantly reduced and the symptom relieved obviously. No symptom recurrence or significant toxicity occurred during or after the treatment. The patient remains under routine follow-up. CONCLUSION: We present a case of a male patient with GPA located exclusively in the eyelid area, who underwent successful radiotherapy and achieved a complete response. The lesson we learned from this case study is that for GPA patients, when the standard treatment model fails to achieve good results, novel treatments such as radiotherapy should be considered according to the situation.

Localized Wegener's granulomatosis.

BACKGROUND: Wegener's granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease. OBJECTIVES: Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG. METHODS: The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied. RESULTS: All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti-neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide. LIMITATIONS: The limited number of patients is counterbalanced by the rarity of the disease. CONCLUSIONS: Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.

Reticulosis letal de la línea media. Manejo con radiaciones y consideraciones histopatológicas / Lethal midline granuloma. Radiotherapy and pathology

Entre 1973 y 1990 se han tratado 24 pacientes portadores de Reticulosis Letal de la Línea Media (RLLM) mediante el empleo de radiaciones ionizantes en el Departamento de Radioterapia del Instituto Nacional de Enfermedades Neoplásicas en Lima, Perú. Los autores realizan una revisión descriptiva y retrospetiva de dichos casos así como una revisión de la literatura referente a este tipo de neoplasia. Todas las láminas provenientes de las biopsias han sido revisadas para confirmar el diagnóstico de RLLM. Todos los pacientes recibieron tratamiento con radiaciones ionizantes y 12 de ellos algún tipo de quimioterapia durante el tratamiento con radiaciones. La relación según sexo masculino-femenino ha sido de 5:3, la edad promedio de 29:33 años y el rango de edad entre los 6 y 84 años de edad. Los síntomas de presentación más comunes han sido: obstrucción de fosa nasal (45.83 por ciento), aumento de volumen de la fosa nasal (33.33 por ciento), rinorrea (29.16 por ciento) y alza térmica constante mayor de 38.5ºC (29.16 por ciento), principalmente. No se ha podido establecer una dosis de referencia para el tratamiento con radiaciones puesto que las dosis han variado desde 732 cGy hasta 7095 cGy; sí hemos podido observar que todos aquellos que recibieron menos dosis de la planificación fue porque fallecieron durante el tratamiento ya que acudieron con enfermedad muy avanzada y en mal estado general. Asimismo, se ha podido corroborar que si la dosis administrada fue de 50 cGy ó más se obtuvo mejor control local aunque por el número de casos no se ha podido demostrar estadísticamente. Al evaluar la sobrevida se observa que ésta disminuye considerablemente durante el primer año de seguimiento pero a partir del segundo año se mantiene constante (33.33 por ciento); esto en relación al estado en que acudió el paciente a recibir tratamiento. Concluimos que en este tipo de neoplasias el empleo de radiaciones ionizantes juega un rol importante en el control local de la enfermedad pero también es importante la extensión de la misma

Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review.

Wegener's granulomatosis is classically a systemic disease which has masqueraded as many other disorders. The patient presenting with head and neck manifestations is often initially referred to the otolaryngologist. Although the natural history of this disease is fairly well understood, its etiology and pathogenesis are not clear. The response of Wegener's granulomatosis to appropriate therapy can be dramatic, whereas without treatment it is a progressive and mutilating disorder with life-threatening complications. Two cases which were recently managed at our center are presented in an attempt to illustrate the complexity of this disorder and the diagnostic and therapeutic dilemma which it presents. A literature review was carried out and a recent concept, the Multistep Evolution Hypothesis, as well as diagnostic advances in serological testing, are discussed. Cyclophosphamide and systemic steroids, the mainstay of therapeutic intervention, as well as more recent modalities including plasmapheresis, folate antagonists, oral trimethoprim-sulfamethoxazole and local radiotherapy are reviewed.

Wegener's granulomatosis. Long-term results of treatment.

Seventeen patients with Wegener's granulomatosis are presented. The very complex clinical features are outlined. A recently suggested new classification (ELK-classification) is applied. Fourteen patients were treated with cytotoxic agents and steroids, 12 of these receiving 6-mercaptopurine. Of the ten patients still alive, eight are in remission with treatment withdrawn in three cases. The duration of the treatment is discussed. The results are largely satisfactory, but the course of the disease is still capricious. Progression to a higher step in the ELK-classification has been observed in several cases and a number of serious sequelae to the disease are recorded. The initial symptoms of Wegener's granulomatosis are varied and uncharacteristic, and it is important to bear this disease in mind when patients with a long course of apparently trivial infections or peculiar constellations of symptoms from several organs are encountered. Repeated biopsies from the respiratory tract are important in order to establish the diagnosis, but treatment should not be delayed in cases where only a tentative diagnosis can be made on the basis of a reasonably typical clinical picture, even with a negative histological response.

Radiation therapy in lymphomatoid granulomatosis.

Two female patients with lymphomatoid granulomatosis have received radiation therapy for local, progressive, symptomatic lesions. One patient was a 56-year-old woman with concomitant brain and pulmonary lesions which progressed on cyclophosphamide and prednisone therapy. The second patient was a 62-year-old woman with a supraorbital mass which appeared and progressed on prednisone therapy. Both patients exhibited rapid response to radiation therapy, in terms of relief of symptoms and objective evidence of subsidence of local disease. A discussion of the nature of lymphomatoid granulomatosis and its treatment is presented with a review of the literature. One hundred sixty-five cases have now been described including the two in this study, and in only one previous case was radiation therapy utilized to treat local disease, prior to the two cases described herein.

[Wegener's granulomatosis. Roentgenographic signs and radiotherapeutic possibilities (author's transl)]. / Wegenersche Granulomatose. Röntgenologische Lungenbefunde und strahlentherapeutische Möglichkeiten.

Pulmonary alterations and the clinical course of 8 patients with Wegener's granulomatosis are described. Knowledge of the variety of the clinical presentation and lung involvement is mandatory for the radiologist to achieve early diagnosis of this disease. Prognosis is better in Wegener's granulomatosis when adequate therapy is started in the first stage. Films of the nasal sinus are also important for the diagnosis. Besides immunsuppressiva radiation therapy can be helpful in local involvement of the ENT, skin, and orbital area as is demonstrated in 2 of our patients.

Midline granuloma and Wegener's granulomatosis: clinical & therapeutic considerations.

Midline granuloma (MG), limited Wegener's granulomatosis (LWG), and generalized Wegener's granulomatosis (WG) have been viewed by some investigators as representing an interrelated disease spectrum. Others believe that MG and WG are two distinct clinicopathologic entities. A series of cases is presented suggesting that therapy of MG should be individualized. Treatment may include corticosteroids, high-dose irradiation, and/or immunosuppressive drugs. LWG may be treated initially with corticosteroids alone, but lack of response requires the addition of an immunosuppressive agent. WG should be treated with an immunosuppressive drug and, at times, corticosteroids as well. None of the cases of MG described in this report progressed to WG. This may be interpreted as supporting the contention that MG and WG are separate diseases. Alternatively, aggressive treatment of MG with irradiation or immunosuppressives may prevent its transition to more generalized disease.