Population-based incidence and survival of gastrointestinal stromal tumours (GIST) in Girona, Spain.

BACKGROUND: Gastrointestinal stromal tumours (GIST) are rare malignancies characterised by their association with KIT oncogene mutations. Until now, population-based reports of the incidence or survival of kit-confirmed GIST have been rare, and none have originated in Southern Europe. MATERIALS AND METHODS: We used the Girona Cancer Registry to identify malignant mesenchymal tumours of the digestive tract between 1994 and 2001, and performed c-kit testing in the tumour samples. Age-adjusted incidence rates and survival rates were calculated, and they were also analysed by sex and NIH risk categories. RESULTS: Forty-six cases were categorised as GIST. Fifty percent were localised in the stomach, 43.5% in small intestine, 4.3% in the omentum, and 2.2% in colon. Thirty-seven percent were classified as high risk of an aggressive behaviour, 30.4% as intermediate risk and 32.6% as low or very low risk. Only one patient received treatment with imatinib mesilate. The annual incidence by 100,000 inhabitants in crude rate, European age-standardised rate and world age-standardised rate was, respectively, 1.09, 0.90 and 0.65 cases. The relative 5-year survival rate was 74.7% for the entire cohort, and it was markedly lower in the high-risk cases (20.3%). CONCLUSIONS: We report the first population-based study of GIST incidence and survival in Southern Europe. The incidence rate is low and comparable with that of cancer registries from Northern Europe. Survival was favourable in our pre-imatinib population although it was low in high risk cases. Prognostic discrimination of the cases with intermediate, low, or very low risk is inadequate, and these categories should be considered jointly in the future. Our results will help researchers in establishing baseline values against which they can compare, in the future, the impact of imatinib and other Kit tyrosine inhibitors on survival.

[Gastrointestinal inflammatory fibroid polyps. Clinical characteristics and follow-up in a series of 26 patients]. / Pólipo fibroide inflamatorio gastrointestinal. Características clínicas y seguimiento de una serie de 26 pacientes.

INTRODUCTION: Inflammatory fibroid polyp (IFP) is a protuberant lesion, located near the muscularis mucosae and composed of a proliferation of fusiform cells and conjunctive fibers surrounding capillaries and a variable inflammatory infiltrate. It is believed to be a poorly controlled inflammatory repair response. Our aim was to study the clinical, pathological and follow-up characteristics of a series of patients with IFP. PATIENTS AND METHOD: We studied 26 IFPs from 25 patients (16 women and 9 men) registered between 1985 and 2001 in a specific register of 3 centers in the city of Gerona (Spain). The variables analyzed were age, sex and clinical presentation, IFP localization and size, mucosal characteristics and associated disease, as well as follow-up information. Routine statistical analyses were performed. RESULTS: IFPs were antral in 16 patients, ileal in 7, jejunal in 2 and colonic in the remaining patient. Size determined whether they were symptomatic (35 +/- 13.6 mm) or asymptomatic (8.4 +/- 6.3 mm). Gastric polyps were significantly smaller than intestinal polyps. Symptomatic polyps (5 out of 16 gastric polyps and 9 out of 10 intestinal polyps) predominated in women and occurred at a significantly lower age than asymptomatic polyps (59.2 versus 74.1 years). Most gastric IFPs were associated with chronic atrophic gastritis while only one ileal polyp was associated with Meckels diverticulum. The mean length of follow-up was 60.6 months and, except in one patient who underwent incomplete resection, no recurrences of IFP were observed. CONCLUSION: IFP is a heterogeneous entity, depending on age at presentation, sex, size and location in the digestive tract. IFP does not recur after resection. The association of gastric IFP and chronic atrophic gastritis could suggest a modulatory effect of the mucosa on IFP growth.

[Immunonegative necrotizing glomerulonephritis: an atypical case of lupus nephropathy]. / Glomerulonefritis necrotizante inmunonegativa: un caso de nefropatía lúpica atípico.

We report here a case of systemic lupus erythematosus with severe, active pauci-immune necrotizing and crescentic glomerulonephritis. This patient had been diagnosed of a lupus nephritis type III previously and treated with steroids and azathioprine. After a renal symptomless period of nine years, she developed heavy proteinuria and hypertension. A second kidney biopsy was then performed. The pathological study disclosed a pauci-immune necrotizing crescentic and segmental glomerulonephritis. The absence of subendothelial and mesangial deposits was confirmed by both immunofluorescent microscopy and electron microscopy. The simultaneous immunological study showed normal ANCA levels while complement, anti-dsDNA and ANA were altered. The patient was treated with steroids and cyclophosphamide eith good response. This case points out the possibility previously recognized by others of an association between lupus and an pauci-immune necrotizing glomerulonephritis.

Fatal right spontaneous haemothorax in Von Recklinghausen's disease.

Spontaneous massive haemothorax is uncommon and usually occurs as a result of pulmonary infarction, arteriovenous fistula, neoplasm, ruptured aortic aneurysm, rupture of pleural adhesions or pleural endometriosis. Massive haemothorax in Von Recklinghausen's disease occurs rarely but with potentially fatal results in spite of surgery. We present a case of a spontaneous massive exsanguinating haemothorax in a patient with neurofibromatosis type 1 caused by rupture of a branch of the right subclavian artery. Bleeding was probably due to neurofibromatous invasion of the arterial wall.

Invisible mycosis fungoides: A diagnostic challenge.

We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.

Fine-needle aspiration cytology of columnar-cell carcinoma of the thyroid: report of two cases with cytohistologic correlation.

The columnar-cell variant of papillary carcinoma is a rare tumor of the thyroid, associated with aggressive behavior, early visceral metastasis, and a rapidly fatal course. In this report we present the fine-needle aspiration cytologic findings of two examples of this variant of papillary carcinoma with cytohistologic correlation. In the smears, clusters, monolayered sheets, and scattered papillary fronds of tumor cells were present. The tumor cells were columnar and exhibited overlapping and stratification of the nuclei. In the first case the tumoral cells showed round nuclei with finely granular chromatin pattern, small nucleoli and vacuolated-appearing cytoplasm. The malignant cells in the second case presented oval to elongated nuclei with stippled chromatin, inconspicuous nucleoli and indistinct cytoplasmic borders. It is important to distinguish this tumor from the common thyroid papillary carcinoma because of its much more aggressive behavior.

Massive crystal-storing histiocytosis associated with low-grade malignant B-cell lymphoma of MALT-type of the parotid gland.

Massive crystal deposition is unusual in lymphoproliferative disorders. In this report, a mucosa-associated lymphoid tissue (MALT) low-grade B-cell lymphoma of the parotid gland containing large numbers of crystal-storing histiocytes is described. The patient, an 81-yr-old female, presented with a history of long-standing left parotid gland enlargement. FNA cytology of the tumor showed a lymphoplasmacytic infiltrate and sheets of large benign histiocytes with abundant eosinophilic intracytoplasmic inclusions. Paraffinsection immunohistochemistry performed on the cell block demonstrated that the histiocytic cells were immunoreactive for the KP-1 (CD-68) antibody and monotypic for cytoplasmic IGM and L-light chain. The cytological diagnosis was consistent with a low-grade B-cell lymphoma with plasmacytic differentiation associated with crystal-storing histiosis. A periparotid lymph node was biopsied and showed involvement by a monocytoid B-cell lymphoma with plasmacytic differentiation and crystal-storing histiocytosis in the pericapsular region.

[Churg-Strauss syndrome: 8 cases in the last 10 years]. / Síndrome de Churg-Strauss. Ocho casos en los últimos diez años.

The clinical and histopathological findings of 8 patients with a diagnosis of Churg-Strauss syndrome seen in our institution in a 10 year period have been reviewed. All patients had asthma and hypereosinophilia (mean eosinophil count 7.64 x 10(9); range: 0.748 x 10(9)-31.46 x 10(9) eosinophils/l). The organs and systems involved in the late phase of the syndrome (vasculitic phase) were: nervous system (16 cases), respiratory system (5 cases), skin and subcutaneous tissue (5 cases), heart (3 cases), digestive system (3 cases) and kidney (1 case). The diagnosis was made on the basis of the clinical and histopathological findings. In 7 cases necrotizing angiitis was shown, tissue eosinophilia in 4, and extravascular granulomas in 2. In most cases, corticosteroid therapy resulted in a favorable course, but cyclophosphamide was required in two patients. In the discussion, the evolution of the criteria for the diagnosis of this condition is analyzed.

Infantile fibrosarcoma of femur.

We describe the clinical presentation and pathological features of an infantile fibrosarcoma of the femur that occurred in a 3 1/2-month-old male infant. The tumor grew rapidly, reaching large size within a few months, finally requiring amputation. The tumor was poorly circumscribed, infiltrating the adjacent soft tissue extensively. Microscopically, it was a highly cellular tumor composed of immature-appearing spindle-shaped cells. Ultrastructural study revealed mesenchymal cells with fibroblastic and histiocytic differentiation.

Nephrogenic metaplasia of the ureter.

A case of nephrogenic metaplasia of the left ureter occurring in a 7-year-old male child, associated with recurrent urinary infection and previous surgical intervention, is described. This is to our knowledge the third reported case of this entity. Nephrogenic metaplasia involves the transitional epithelium of the urinary tract and results in the formation of structures histologically similar to renal tubules.

Amiodarone and pulmonary fibrosis.

Of 25 cases of idiopathic pulmonary fibrosis, confirmed by open chest lung biopsy between January 1979 and July 1981, 5 patients had been taking amiodarone in doses less than 600 mg daily for 1 to 6 years. No lung disease had been observed in any patient before the administration of amiodarone, and other drugs, factors and diseases thought to play a role in the causation of pulmonary fibrosis were ruled out. Two patients died of respiratory insufficiency, and only 1 does not now suffer from serious sequelae. Review of a further 5 cases reported in the literature and the present data suggest a causal link between administration of amiodarone and pulmonary fibrosis, and tend to indicate a hypersensitivity mechanism.

[Chronic eosinophilic pneumonia: report of eight cases (author's transl)]. / Neumonía eosinófila crónica. Estudio de ocho casos.

Eight cases of chronic eosinophilic pneumonia (CEP) diagnosed between January 1977 and December 1979 are described. Clinical manifestations included toxic syndrome, cough and fever, lasting from 1 to 15 months. Chest x-ray revealed peripheric bilateral infiltrates, with the exception of one case. In two patients there was no peripheral eosinophilia and five received antituberculous drugs at some point during the illness. In all cases tests for fungi and parasites were negative. In only two patients was an increase in IgE found. Hystological study confirmed CEP in 7 patients through either trans-bronchial biopsy or minimal thoracotomy. Treatment with corticosteroids was dramatically effective in all patients; both clinically and radiologically. In two cases which were asymptomatic, decreased carbon monoxide diffusing capacity persisted six months later. One hundred fifteen cases of CEP published since Carrington et al. first described CEP as a separate entity of the pulmonary infiltrates with eosinophilia syndrome are reviewed.

[Granulomatous hepatitis: aetiological study of 107 cases (author's transl)]. / Hépatite granulomateuse: étude étiologique de 107 cas.

In this series, the commonest aetiology was tuberculosis (30 cases, 28%), followed by sarcoidosis (18 cases, 17,7%), mediterranean fever (Olmer's disease) (13 cases, 12,1%), brucellosis (8 cases, 7,4%), typhoid fever (7 cases, 6,6%) and idiopathic forms (8 cases, 7,4%). These were followed by Hodgkin's disease, toxoplasmosis, adenosarcoma, and leprosy. Finally, there were single cases due to infectious mononucleosis, B.C.G. reaction, hypogammaglobulinaemia, coeliac disease and temporal arteritis. Half of the patients had hepatomegaly and an increase, in general moderate, in hepatic enzymes (transaminases, alkaline phosphatase). The highest enzyme levels were seen in cases of brucellosis, hepatic enzymes being normal in patients with sarcoidosis.