Cases reported "Recurrence"

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1/202. fever of unknown origin due to inflammatory pseudotumour of lymph nodes.

    The clinical features of inflammatory pseudotumour of lymph nodes, a distinct non-malignant histopathological entity firstly described in 1988, are summarized based upon a detailed analysis of 4 personal cases and 47 cases reported in the literature. The mean age of the patients is 38 years (range 8 to 82 years) and there is no gender predilection. One third present with asymptomatic lymphadenopathy and 47% present with fever, nearly all meeting the criteria of fever of unknown origin. Abdominal complaints are occasionally present. Intermittence of symptoms is common. Hepatosplenomegaly is unusual. All lymph node areas may be involved but abnormalities are mostly confined to one or two anatomic regions. No extranodal involvement has been reported although inflammatory pseudotumour may occur in several organs with similar morphological features and identical signs of inflammations. Routine blood tests are normal except for signs of inflammation. The lesions are gallium-avid. diagnosis is based upon typical histopathological features. The prognosis is favorable and surgical resection frequently leads to cure. Spontaneous resolution of symptoms has been reported and nonsteroidal anti-inflammatory drugs may suppress the clinical manifestations.
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2/202. Successful salvage treatment with irinotecan (CPT-11) of recurrent malignant lymphoma in an aged patient; and CPT-11 pharmacokinetics.

    A 72-year-old patient with relapsed non-Hodgkin's lymphoma (diffuse large B-cell type) in the tongue was treated with irinotecan (CPT-11) as the 4th salvage therapy. A two-thirds reduced dose of 40 mg/m2 of CPT-11 was administered, as were granulocyte-colony stimulating factor and antidiarrheal agents. Complete remission was achieved. Although grade 3 leukopenia and grade 1 diarrhea were observed, these adverse reactions did not interrupt the treatment schedule and CPT-11 was administered without interruption for a total of 12 weeks. Despite the dose reduction, the area under the concentration-time curve of SN-38, the active metabolite of CPT-11, was nearly equal to the values reported in phase I and II studies of CPT-11. The patient's ratio of SN-38 to the SN-38 glucuronide (SN-38G) was low, suggesting a low risk of diarrhea. The optimal dose modification provided a sufficient amount of the active metabolite. Supportive therapy managed therapy-related toxicities and resulted in a stable treatment schedule. This is a rare case of a patient successfully treated with CPT-11 after a 4th relapse, in which the agent was administered at the total dose of 960 mg/m2, despite the patient's advanced age.
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3/202. A case of Crohn's disease with recurrent massive life-threatening hemorrhage from terminal ileum.

    A case of Crohn's disease that underwent bowel resection two times at 3-year intervals due to life-threatening hemorrhage from ileum is presented. The bleeding sites were located in the ulcer lesions of ileum, in the oral side near to the ileum-colon transition. The first bleeding point was at the longitudinal ulcer of the terminal ileum and the secondary bleeding site was at the profound ulcer of ileum appearing in the oral side near to the ileo-colic anastomosis. This is the first case of Crohn's disease with recurrent life-threatening massive hemorrhage in the terminal region of ileum, for which operative bowel resections were necessary. This case suggests that we should search for the bleeding site in ileal lesions developed in the circumference of and especially the oral side near to the anastomosis due to prior resection, when intestinal massive bleeding occurs again after bowel resection, and that the careful follow-up and strict treatment with diet therapy and/or anti-inflammatory drugs are necessary for the protection of recurrence in patients with Crohn's disease.
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4/202. Benefits of endorectal ultrasound for management of smooth-muscle tumor of the rectum: report of three cases.

    Smooth-muscle tumor of the rectum is rare, and the therapeutic strategy is still controversial. Endorectal ultrasound was used to evaluate three patients with smooth-muscle tumor of the rectum. Endorectal ultrasound demonstrated a homogenous hypoechoic tumor without invasion to the perirectal tissue in two patients. The tumor was 5 cm in diameter in one patient and 4 cm in diameter in the other patient, and they were excised locally. Their histologic types were leiomyoma and leiomyosarcoma. The third patient had a recurrent leiomyosarcoma. Proctosigmoidoscopy found a linear lesion with ulcerated mucosa on the rectal wall. Endorectal ultrasound observed a hypoechoic solid tumor of 3.5 cm x 1 cm, which involved the mucosal, submucosal, and muscle layers of the rectal wall. Disruption of the first hypoechoic layer was identified. Abdominoperineal resection was performed. Endorectal ultrasound follow-up revealed no evidence of recurrence in any of these patients. Endorectal ultrasound can help to define the extent of disease and may be a useful adjunct in deciding about the appropriate surgical procedure in these diseases.
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5/202. Recurrent Goodpasture's disease due to a monoclonal IgA-kappa circulating antibody.

    We describe the case of a 54-year-old man who first presented with a clinical syndrome manifested by recurrent pulmonary hemorrhage, hematuria, and mild renal insufficiency. Direct immunofluorescence of renal biopsy sections showed linear deposition of IgA-kappa in the glomerular (GBM) and tubular basement membranes. serum protein immunoelectrophoresis was positive for a monoclonal immunoglobulin A (IgA)-kappa protein. serum analysis showed circulating IgA anti-GBM antibodies. Treatment with high-dose steroids, cyclophosphamide, and plasma exchange resulted in resolution of the clinical picture. To the best of our knowledge, this is the first report of Goodpasture's disease associated with the presence of a circulating monoclonal IgA-kappa antibody.
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6/202. Antegrade recanalization of completely embolized internal carotid artery after treatment of a giant intracavernous aneurysm: a case report.

    BACKGROUND: Parent artery occlusion is one of the traditional methods of treatment for unclippable aneurysms. However, parent artery occlusion may not result in permanent exclusion of the aneurysm from the systemic circulation. We present a case of cerebral aneurysm treated by proximal embolization of the parent artery, which recanalized during the follow-up period. CASE DESCRIPTION: A 69-year-old woman presented with a right blepharoptosis and diplopia. A large aneurysm arising from the cavernous portion of the right internal carotid artery was found and endovascularly excluded from the cerebral circulation by proximal internal carotid artery occlusion with balloons. Eleven days after treatment, occlusion of the parent artery and obliteration of the aneurysm were angiographically confirmed. However, the parent artery was found to be recanalized with nearly total obliteration of the aneurysm at the follow-up 6 months after treatment. CONCLUSION: angiography suggested that recanalization took place through the vaso vasorum. We believe that recanalization was induced by marginal cerebral blood flow in the ipsilateral hemisphere.
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7/202. Histologically repeatedly confirmed gliosarcoma with long survival: review of the literature and report of a case.

    OBJECTIVE AND IMPORTANCE: A rare case of gliosarcoma in a 61-year-old woman is presented with a stable situation over 22 years with an excellent quality of life. CLINICAL PRESENTATION: The patient was initially symptomatic and was operated on in 1975 for a deep-seated left parietal gliosarcoma. During the following 20 years, she was clinically asymptomatic until she complained of increasing headache in 1995. Neuroradiological imaging showed a sharply demarcated lesion on MRI at the former operative site, which was operated on again. Four months later, the residual tumour did grow again. INTERVENTION: As radiation therapy could not stop tumour progression and the neurological status worsened, the patient was operated on again for a massive tumour mass in the left parieto-occipital region, filling out nearly all of the previous resection cavity. Despite radio-immunotherapy, the patient finally died 22 years after the first discovery of the tumour. CONCLUSION: The present case shows that, in rare instances, gliosarcomas may show prolonged survival, although the underlying pathogenetic mechanisms for this clinical behaviour are not understood.
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8/202. Generalized pruritus without primary lesions. Differential diagnosis and approach to treatment.

    A 65-year-old man presented with recurrent generalized pruritus and excoriations of many years' duration. He had been treated with antihistamines, topical corticosteroids, and antibiotics for secondary wound infections, but improvement was only temporary. He had also been hospitalized for leg ulcers complicated by cellulitis. Examination revealed multiple oval and linear red papules and nodules measuring 0.5 to 2 cm in diameter. Some of the lesions were eroded and had a central crater and yellowish crust. The patient also had hypopigmented linear scars localized to the posterior scalp, neck, upper back, chest, abdomen, arms, and legs with sparing of the middle and lower back (figures 1 and 2). An ulcer measuring 1.5 x 2 cm that was surrounded by indurated skin was present on the medial aspect of his right ankle. The ulcer was partially covered by yellow exudate. There was no evidence of cellulitis. liver enzyme, serum creatinine, and thyrotropin levels, as well as a chest roentgenogram, were normal. Wound cultures for bacteria and fungi were nonsignificant. A punch biopsy from a representative lesion showed an abrupt epidermal defect with sparse superficial lymphocytic infiltrate in the dermis. The patient was admitted to the hospital to isolate him from his home environment. He received a 10-day course of systemic cephalexin, topical clobetasol propionate ointment for the affected skin areas, and oral hydroxyzine for pruritus. Ultraviolet light therapy was instituted once daily and was to continue for 2 months. His lesions had improved moderately by the time he was discharged from the hospital. On follow-up 2 weeks later, his lesions were flat and had resulted in hypopigmented scars. Three months later, however, he had persistent, intense pruritus, and new excoriations had developed on his forearms and back. He improved after receiving treatment with oral doxepin hydrochloride.
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9/202. Progressive pedal macrodactyly surgical history with 15 year follow-up.

    Macrodactyly can affect the fingers and/or toes1. Histopathologic examination will distinguish macrodactylia fibrolipomatosis or neural fibrolipoma with macrodactyly, from macrodactylia as a part of neurofibromatosis. Surgical repair is aimed at decreasing the size of the affected foot so it is as near in size and shape to the normal foot as possible. Surgical approaches have included reconstructive surgery (usually staged debulking procedures), epiphyseal plate arrest and amputation. Repeated reconstructive surgical procedures, as illustrated in this report covering patient care over a 15 year period, are usually necessary due to recurring soft tissue and boney enlargement.
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10/202. Type 2 segmental manifestation of Hailey-Hailey disease: poor therapeutic response to dermabrasion is due to severe involvement of adnexal structures.

    In autosomal dominant skin conditions, two different types of segmental manifestation can be distinguished. Type 1 represents heterozygosity for a postzygotic mutation, resulting in a degree of severity similar to that of the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and shows an excessively pronounced involvement superimposed on the ordinary nonsegmental phenotype. We describe the clinical, histopathological and therapeutic aspects of the first case of type 2 segmental manifestation of Hailey-Hailey disease (HHD). A 24-year-old woman with a family history of HHD comprising four generations, presented with lesions of erythema and blistering arranged in a unilateral pattern following the lines of Blaschko. The disorder was first noted at the age of 3 months. At the age of 24 years, additional scattered symmetrical lesions involving the axillary and inguinal folds were noted. Histopathological examination of the severely involved linear skin areas revealed pronounced acantholysis within the deep adnexal structures, whereas clinically unaffected skin showed the typical histopathological features of the heterozygous phenotype with suprabasal clefting and acantholysis sparing the adnexae. dermabrasion was performed in the areas of segmental involvement. During a follow-up period of one year, no recurrence was noted, but 18 months after dermabrasion a recurrence was present in the left submammary and left perianal regions. This therapeutic resistance to dermabrasion may be explained by the presence of acantholysis within the adnexal structures of the skin as found in type 2 segmental HHD.
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