[Malignant fibrous histiocytoma of soft tissue: description of 10 cases]. / Histiocitoma fibroso maligno de partes blandas: descripción de 10 casos.

We analyze the clinical and histological features of 10 cases of malignant fibrous histiocytoma of soft tissue. Nine belonged to the pleomorphic-verticillate variety and one was myxoid. The initial clinical feature was a palpable mass in all cases except three with retroperitoneal localization, where constitutional symptoms predominated. After therapy (surgery in all, associated with radiotherapy in four), seven patients had local relapse and two had distant metastases. 50% died, with a mean survival of 13 months. We discuss the prognostic factors and the therapeutic approach, with emphasis on aggressive therapy and the need for radical surgery and postoperative adjuvant therapy.

[Systemic mastocytosis. Study of 14 cases]. / Mastocitosis sistémica. Estudio de 14 casos.

We have analyzed the clinical, analytical and evolutive data of 14 cases of systemic mastocytosis (SM) diagnosed in our hospital between 1991-1996 and we have compared these results with those of other published series. Clinical parameters, analytical profiles, peripheral hematologic data, radiologic data and histological study were collected. Following Metcalfe's criteria, the patients were classified in 4 groups: a) group 1, indolent mastocytosis; b) group 2, hematologic disorders; c) group 3, aggressive lymphadenopathic mastocytosis with eosinophilia, and d) group 4, mastocytic leukemia. The average age at diagnosis was 52.4 years, range 25-83, and 64% were females. The mean follow-up was 2 yr. In most of the cases (71.4%) the initial complaint was urticaria pigmentosa. The predominant clinical features were pruriginous-eritematous skin lesions (in 11 cases), and digestive symptoms (in 10 patients). The most usual biochemical disorder was the rise of serum alkaline phosphatase level (in 8 patients), while lactate-dehydrogenase (LDH) was normal in all the cases. The most striking roentgenologic features were oteopenia, observed in 50% of our patients. Pheripherical hematological disorders were discovered in 8 patients (64.3%) and in one of them circulating mast-cells were observed. The bone marrow was involved in all patients (100%) and in two of them mielodysplasic features were found. The diagnostic of SM is difficult in the absence of skin lesions. The skin lesions are very common in systemic mastocytosis. Bone marrow involvement is constant, so its study has a high diagnostic rentability.

[Wegener's granulomatosis with meningeal involvement: clinic-radiological correlations]. / Granulomatosis de Wegener con afectación meníngea: correlación clinicorradiológica.

INTRODUCTION: Wegener's granulomatosis is a systemic vasculitis which, in its classical form, is characterized by involvement of the superior and inferior respiratory tract and the kidneys. The vasculitis may be multisystemic. Ophthalmic and neurological involvement are common (22% and 54% of those affected respectively). When considering involvement of the nervous system, the commonest finding is peripheral neuropathy, particularly in the form of multiple mononeuritis. Meningeal involvement is exceptional. CLINICAL CASE AND CONCLUSIONS: We present a case of Wegener's granulomatosis with meningeal involvement, studied using CT and MR. The findings using imaging techniques are described, and conditions which should be considered in the differential diagnosis are discussed.

[Peripheral neuropathy in systemic lupus erythematosus]. / Neuropatía periférica en el lupus eritematoso sistémico.

INTRODUCTION: Systemic lupus erythematosus (SLE) is the connective tissue disorder in which neuropsychiatric complications are most often seen. The prevalence of peripheral neuropathy varies from 5 to 27% depending on the series of cases described, with subclinical forms being most frequent. OBJECTIVES: To determine the prevalence of subclinical peripheral neuropathy in a group of patients with SLE, define their characteristics, and investigate any possible associations with clinico analytical parameters of the disease. PATIENTS AND METHODS. 32 patients with SLE were diagnosed in an Internal Medicine Outpatient Clinic and followed up for at least a year. They were studied prospectively and consecutively by clinical examination, laboratory test and neurophysiological studies (electroneurogram). RESULTS: Neurophysiological study showed peripheral neuropathy to be present in 50% of the patients, who were mainly asymptomatic (75%) and found to be normal on neurological examination (81.2%). The most frequent type of neuropathy was polyneuropathy (37.5%) which was predominantly axonal symmetrical and sensomotor followed by isolated mono neuropathy (6.2%) and multiple mononeuritis (6.2%). No clinical or analytical parameter was significantly associated with the development of peripheral neuropathy. CONCLUSIONS: The considerable frequency of subclinical peripheral neuropathy in patients with SLE and the absence of associated clinical or analytical parameters makes it necessary to carry out neurophysiological studies in these patients to detect its presence and establish the precise extent of the disorder.

[Diabetes mellitus and pancreatic tumor]. / Diabetes mellitus y tumor pancreático.

A 68 year old Ecuadorian man was investigated for polyuria, polydipsia and weight loss of 3 kg during the previous two months. Insulin dependent diabetes mellitus was diagnosed 10 year before admission and treated with appropriate diet and insulin (35 U/d). 18 months before was diagnosed in El Ecuador of "multiple liver nodes non-suggestive of malignancy". Physical examination showed a large multinodular petrous hepatomegaly. There was no evidence of skin lesions. Results of laboratory studies included a basal plasma glucose level that ranged between 275-367 mg/dl (N=60-100), glycosylated haemoglobin of 8.9% (N<5) and a serum albumin of 2.8 gr./dl (N=3.4-4.8). At admission non-other laboratory alterations were detected. Computed tomography showed a mass on the head of the pancreas with loco-regional lymph nodes and liver metastases. Tumor markers were normal. Fine-needle aspiration cytology of the liver masses revealed the presence of liver metastases of a non-differentiated malignant tumor. A 111In-DTPAOC scintigraphy revealed the presence of somatostatin receptors in the liver metastases, also detecting the presence of multiple bone metastases in the axial and appendicular skeleton. Plasma glucagon level was 678 pg/ml (N<250). A diagnosis of metastatic glucagonoma was established and therapy with streptozocin, 5-FU, insulin and synthetic somatostatin analogs was initiated. Three months after the therapy initiation the patient was symptom free. Some weeks after the patient suffered from left hip pain, and a control 111In-DTPA scintigraphy showed progression of his bone metastases. In conclusion, glucagonoma must be suspected in all diabetic patients with metastatic liver, even in absence of necrotic migratory erythema. In these circumstances, plasmatic glucagon level and somatostatin receptors scintigraphy will be a useful tool for establishing the final diagnosis.

[Carcinoid tumor and bone metastases: diagnosis by somatostatin receptor scintigraphy]. / Tumor carcinoide y metástasis óseas: diagnóstico mediante gammagrafía de receptores de somatostatina.

OBJECTIVE: The aim of this study has been to retrospectively assess the usefulness of 111In-DTPAOC scintigraphy in the detection of bone metastases (BM) in patients diagnosed of carcinoid tumour (CaT). MATERIALS AND METHODS: Between June 1995 and April 2003 78 111In-DTPAOC studies were consecutively performed in 58 patients, 31 females and 27 males, 28 to 73 years old, with a histological diagnosis of CaT. Moreover, whole body bone scans (BS) using 99mTc-MDP were performed in 13 of these patients. The patients were classified into three groups: Group A: Initial CaT staging (n = 23); Group B: CaT staging after surgery (n = 14); and Group C: Post-treatment CaT re-staging (n = 29). In this last group, 6 patients of group A and 2 patients of group B were included. In only 2 patients the diagnoses of bone metastases were established before the 111In-DTPAOC scan. RESULTS: Twenty six (44.8 %9 of the 58 patients with CaT had metastatic disease: 15 patients with hepatic metastases, associated with BM in 4 of them, 10 patients with hepatic and extra-hepatic metastases, abdominal and/or thoracic, associated with BM in 4 and in one patient, the BMs were the only metastases detected. The global incidence of BM in patients diagnosed with CaT was 15.5 % (9/58), whereas the incidence of BM in patients with metastasic disease was 34.6 % (9/26). Significant differences (p = 0.0035) were found on the incidence of BM in patients with or without hepatic metastases. In 4 patients, BMs were detected during the initial staging (group A), whereas in 5 patients, BMs were detected during the post-treatment re-staging (group C). During diagnosis, 4 of the 9 patients with BM had bone pain. BM were multiples in 8 patients, affecting axial skeleton in 4 and axial and appendicular skeleton in 4. One patient had a diffuse infiltration of bone marrow. BS was positive in 8 of the 9 patients with BM. In these 8 patients with abnormal BS, 111In-DTPAOC scintigraphy provides similar information to the BS in one patient, shows a greater number of bone lesions in 3, whereas BS was superior in 5 patients. Four of the patients with BM died between 6 and 47 months after diagnosis (mean: 29.7 months). CONCLUSIONS: BMs are preferably located on axial skeleton, can be asymptomatic and are associated with hepatic metastases. Although the 111In-DTPAOC scintigraphy is able to detect some BM earlier than BS, the information provided by both studies is complementary. In patients with CaT, any invasive therapy on the hepatic metastases make it necessary to exclude extrahepatic metastases, including bone ones, and the somatostatin receptor scintigraphy is the diagnostic method of choice.

[18F-FDG PET/CT diagnosis of liver cyst infection in a patient with autosomal dominant polycystic kidney disease and fever of unknown origin]. / Diagnóstico mediante 18F-FDG PET-TAC de infección quística hepática en paciente con enfermedad poliquística renal autosómica dominante y fiebre de origen desconocido.

The diagnosis, localization and treatment of infected cysts in the kidney or liver of patients with autosomal dominant polycystic kidney disease (ADPKD) remain a clinical challenge. We report the findings of (18)F-FDG PET-CT in an ADPKD diagnosed patient who required renal transplantation five years before and in his follow up presented repeated episodes of bacteriemia without known focus on radiological tests performed. The (18)F-FDG PET-CT scan showed numerous hypermetabolic images with focal or ring-shaped morphology related to the content and the wall of some hepatic cysts. The increased metabolic activity was localized on segments VI and VII. We proceeded to drainage of one cyst in segment VI, removing 110 cc of purulent fluid which grew E. Coli BLEE. The (18)F-FDG PET/CT scan should be included in the diagnostic algorithm for detecting infected liver cysts in patients with ADPKD and fever of unknown origin.

[Resistance to first line chemotherapy treatment in a patient diagnosed of nodular lymphocyte-predominant Hodgkin's lymphoma identified by 18F-FDG PET/CT: diagnostic and therapeutic implications]. / Resistencia al tratamiento de primera línea de quimioterapia en un paciente diagnosticado de linfoma de Hodgkin de predominio linfocítico nodular identificada mediante (18)F-FDG PET/TAC: implicaciones diagnósticas y terapéuticas.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare entity that accounts for less than 5% of the cases of Hodgkin lymphoma (HL) with morphological, immunophenotypical, genetic and clinical behavior traits different from the classic HL. In a minority of patients, the NLPHL course is complicated by a transformation to a non-Hodgkin diffuse large B-cell lymphoma (NHDLBCL) with prognostic and therapeutic implications. Early metabolic changes observed by (18)F-FDG PET in patients with HL and NHL, after 1-3 cycles of chemotherapy, predict the final response to treatment and progression-free survival. In the case we are presenting herein, whether NLPHL is transformed to NHDLBCL or the two types of lymphoma co-exist in the same patient, the (18)F-FDG PET/CT scan was crucial for the identification of tumor resistance to first line chemotherapy and to guide a second biopsy decision and therefore modify the chemotherapy regimen.

Eosinophilic infiltration of the gastro-intestinal tract (eosinophilic gastroenteritis).

Two further cases of eosinophilic infiltration of the gastro-intestinal tract (eosinophilic gastroenteritis) are presented, involving the small intestine of a 73-year old male and the stomach of a 39-year old male. Peripheral blood eosinophilia was present in one case. Clinico-pathological correlations are discussed.

[Exophthalmos and cranial neuropathy as a form of presentation of Wegener's granulomatosis]. / Exoftalmos y neuropatía craneal como forma de presentación de la granulomatosis de Wegener.

The cases of three patients with Wegener's granulomatosis presenting as exophthalmos and cranial neuropathy are reported. In the first patient's symptoms were secondary to the presence of a retro-orbital mass. In the second and third patients there was dissemination of neighboring granulomatous processes. Paranasal sinuses were involved in two patients, but clinical manifestations were evident in only one. Full remission was achieved in all three. Two patients had been received immunosuppressant therapy for inflammatory processes evaluated a posteriori in the context of Wegener's granulomatosis. The immunosuppressant treatment seems to have conditioned this unusual clinical presentation.

Diagnóstico mediante 18F-FDG PET-TAC de infección quística hepática en paciente con enfermedad poliquística renal autosómica dominante y fiebre de origen desconocido / 18F-FDG PET/CT diagnosis of liver cyst infection in a patient with autosomal dominant polycystic kidney disease and fever of unknown origin

El diagnóstico, la localización y el tratamiento de los quistes renales o hepáticos infectados en pacientes con enfermedad poliquística renal autosómica dominante (EPRAD) sigue siendo un reto clínico. Comunicamos los hallazgos de la 18F-FDG PET-TAC en un paciente diagnosticado de EPRAD, trasplantado renal hace 5 años, que presentó episodios repetidos de bacteriemia sin foco conocido en las exploraciones radiológicas practicadas. La exploración con 18F-FDG PET-TAC demostró numerosas imágenes hipermetabólicas de morfología focal o anular relacionadas con el contenido y la pared de alguno de los quistes hepáticos. La mayor actividad metabólica se localizó en los segmentos vi y vii . Se procedió a la punción y drenaje de uno de los quistes del segmento vi extrayendo 110 cc de un líquido purulento en el que creció Escherichia coli (E. coli) BLEE. La exploración de la 18F-FDG PET-TAC debería incluirse en el algoritmo diagnóstico para detectar quistes hepáticos infectados en pacientes con EPRAD y fiebre de origen desconocido(AU)

The diagnosis, localization and treatment of infected cysts in the kidney or liver of patients with autosomal dominant polycystic kidney disease (ADPKD) remain a clinical challenge. We report the findings of 18F-FDG PET-CT in an ADPKD diagnosed patient who required renal transplantation five years before and in his follow up presented repeated episodes of bacteriemia without known focus on radiological tests performed. The 18F-FDG PET-CT scan showed numerous hypermetabolic images with focal or ring-shaped morphology related to the content and the wall of some hepatic cysts. The increased metabolic activity was localized on segments VI and VII. We proceeded to drainage of one cyst in segment VI, removing 110 cc of purulent fluid which grew E. Coli BLEE. The 18F-FDG PET/CT scan should be included in the diagnostic algorithm for detecting infected liver cysts in patients with ADPKD and fever of unknown origin(AU)

Resistencia al tratamiento de primera línea de quimioterapia en un paciente diagnosticado de linfoma de Hodgkin de predominio linfocítico nodular identificada mediante 18F-FDG PET/TAC: implicaciones diagnósticas y terapéuticas / Resistance to first line chemotherapy treatment in a patient diagnosed of nodular lymphocyte-predominant Hodgkin's lymphoma identified by 18F-FDG PET/CT: diagnostic and therapeutic implications

El linfoma de Hodgkin de predominio linfocítico nodular (LHPLN) es una rara entidad que representa menos del 5% de los casos de linfoma de Hodgkin (LH) con rasgos morfológicos, inmunofenotípicos, genéticos y de comportamiento clínico distintos del LH clásico. En una minoría de pacientes, la evolución del LHPLN se complica por transformación a un linfoma no Hodgkin difuso B de células grandes (LNHDBCG) con implicaciones pronósticas y terapéuticas. Los cambios metabólicos precoces observados mediante 18F-FDG PET en pacientes con LH y LNH, después de 1-3 ciclos de quimioterapia predicen la respuesta final al tratamiento y la supervivencia libre de progresión. En el caso que presentamos, tanto si se trata de LHPLN transformado a LNHDBCG o la coexistencia de los dos tipos de linfoma en el mismo paciente, la exploración 18F-FDG PET/TAC fue determinante para identificar la resistencia del tumor a la primera línea de quimioterapia, orientar la toma de una segunda biopsia y modificar el régimen de quimioterapia(AU)

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare entity that accounts for less than 5% of the cases of Hodgkin lymphoma (HL) with morphological, immunophenotypical, genetic and clinical behavior traits different from the classic HL. In a minority of patients, the NLPHL course is complicated by a transformation to a non-Hodgkin diffuse large B-cell lymphoma (NHDLBCL) with prognostic and therapeutic implications. Early metabolic changes observed by 18F-FDG PET in patients with HL and NHL, after 1-3 cycles of chemotherapy, predict the final response to treatment and progression-free survival. In the case we are presenting herein, whether NLPHL is transformed to NHDLBCL or the two types of lymphoma co-exist in the same patient, the 18F-FDG PET/CT scan was crucial for the identification of tumor resistance to first line chemotherapy and to guide a second biopsy decision and therefore modify the chemotherapy regimen(AU)