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P0273 MESENTERIC TUMOR. DIFFERENTIAL DIAGNOSIS AND MEDICAL TREATMENT OF SCLEROSING MESENTERITIS-MESENTERIC PANNICULITIS
Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283 P0272 ERYTHEMA NODOSUM AND ALCOHOL ABUSE IN REMISSION
Eduardo Santos Ribeiro, Dulcídia Sá, Zoya Klitna, Sandra Rodrigues, Rosa Jorge. Hospital Infante D. Pedro, E.p.e. - Aveiro, Portugal - Serviço De Medicina Ii Erythema nodosum (EN) is characterized by red or violet subcutaneous nodules that usually develop in a pretibial location and usually is associated with infections, drugs, sarcoidosis, collagen diseases, inflammatory bowel disease and other. The authors present a 40 years old patient, with history of alcohol abuse till about 1 year ago, pre-medicated with thiapride, oxazepam and oral contraceptive that came into the Emergency Room because of fever (37.9°C), pitting edema of the legs and erythematous eruptions since 3 weeks. On physical examination, the patient was afebrile, presenting with pitting edema of the legs and with bilateral, painful, erythematous, maculopapular eruptions, suggestive of erythema nodosum, without other relevant changes on physical exam. The laboratory showed ESR 45 mm (<25), CRP 11.78 mg/dL (<0.5), ADA 29.3 IU/L (4.8 – 23.1), SACE 84.3 IU/L (<40), negative ANA, ENA and ANCA, negative HIV, HCV and HBV serologies, negative ASO titer and blood culture. The Mantoux test and the Koch’s bacillus culture were also negative. CT scan showed multiple mediastinal adenopathies whose histologic examination made the diagnosis of sarcoidosis. Then was performed a fiberoptic bronchoscopy and bronchoalveolar lavage that showed a CD4/CD8 ratio of 5. The DLCO was normal. After 1 year of follow-up the patient has no symptoms and the SACE decreased to 45 IU/L (<40), without any therapy. Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that affects individuals worldwide and is characterized pathologically by the presence of noncaseating granulomas in involved organs. The thorax is involved in up to 90% but it can also affects the skin, eyes, heart, CNS, bones and joints. The prognosis of sarcoidosis is generally favourable. Complete spontaneous remission is seen in 60-80% and treatment should be given only to patients with maintained symptomatology, progressive lung disease, eye, CNS or heart involvement and malignant hypercalcemia.
P0273 MESENTERIC TUMOR. DIFFERENTIAL DIAGNOSIS AND MEDICAL TREATMENT OF SCLEROSING MESENTERITIS-MESENTERIC PANNICULITIS
Jose Manuel Rodriguez-Chinesta 1 , Fernando Cibrian 2 , Fernando Garcia-Ruiz 1 , Milagros Garcia 1 , Juan Diez 1 . 1 Usp Clinica La Esperanza. Vitoria-gasteiz; 2 Residencia Arana. Diputación De Alava Introduction: Sclerosing mesenteritis-mesenteric panniculitis is a chronic inflammatory unspecific process which involves mesentery, and its diagnosis must be performed by histology. It is a quite rare condition of unknown etiology. We report a new case and make a differential diagnosis with other inflammatory and tumoral diseases which affect to mesentery, epiplon and bowel. Case report: Woman of 25 with nor previous diseases of interest neither toxic habits. During the three months prior to admission at the hospital she began to feel a non-acute abdominal pain on the left hypochondrias, constipation and sometimes an acute abdominal pain after eating. The physical examination showed just a pain in the mesogastrio and left hypochondrias. A blood sample for a general analysis and serologies was normal. No tumoral markers were found. A chest X-ray and an intestinal transit were normal A mesenteric tumour of 7.5×6.2×6 cm. appeared in a CT and MNR scan. The tumour displaced stomach, pancreas and transverse colon. The mass had not calcium spitting inside. No regional adenopaties were found. After the discovering of the tumour the patient went into a laparotomy Then a mesenteric tumour could be seen which infiltrated stomach, pancreatic itsmo, transverse colon, mesocolon and small bowel mesentery. The mass included the superior mesenteric artery and vein. The microscopic exam with inmunohistochemist and PAS techniques was suitable for a sclerosing mesenteritis. No malignancy was observed. There was a marked inflammatory infiltrate. The patient received metilprednisolone bolus during three days followed by oral prednisone and colchicine. The symptoms disappeared four days after the beginning of treatment. Discussion & conclusion: This case raises the differential diagnosis with
S95
desmoids fibromatosis which has not the inflammatory component. We can also make a differential diagnosis with inflammatory pseudotumour actine-positive, and with inflamatory liposarcoma which is (as in our case) positive for S100 proteine but has atipic and multinucleous cells. Besides the liposarcomais a good definited mass which can be easily removed.
P0274 CASTLEMAN DISEASE: A CLINICAL AND HYSTOLOGICAL STUDY OF THREE CASES
Maria Fernandez Cardona, Margarita Ganzo Pion, Belen Macias Bou, Itxasne Cabezon Estevanez, Cristina Diez Romero, Paloma Diez Romero, Alejandro Fernandez Simon, Isabel Perez Tamayo, Miguel Angel Perez Tascon, Francisco Javier Torres Segovia. H.g.u.gregorio Marañon, Medicina Interna 3 Introduction: Castleman’s disease is a lymphoproliferative disorder associated with HIV, HHV8 and a number malignancies including Kaposi’s sarcoma and lymphoma. It can be unicentric (lymph nodes localized in head-neck and thorax) or multicentric (disseminated lymph nodes, hepatosplenomegaly, constitutional symptoms and laboratory abnormalities). There have been described three hystological variants: hyaline vascular, plasma cell and plasmablastic variant. A wide broad of therapies have been tried, including surgery, radiation, steroids, antiviral agent, specific antibodies, agents that inhibit cytokine activity, as well as chemotherapy. Objectives: The purpose of this study is to show the clinical presentation of an unsual hematological pathology and its therapeutical management. Materials & methods: In this descriptive study we recruited the pacients that presented pathological adenopaties at the admission in our hospital between January 2003 and December 2008. Results: A total of 595 patients diagnosed of pathological adenopaties were studied, three of them were diagnosed of Castleman’s disease. Two cases presented multicentric variant. Two of them were associated with HHV8, one of them with Kaposi’s sarcoma. Case 1
Case 2
Case 3
+ – – – – – + +
+ + + + + + + +
+ + + + + + + +
Lymph nodes Hepatoesplenomegaly Fever Weight loss Hypoalbuminemia Pancytopenia Hypergammaglobulinemia Increased erytrocythe sedimentation rate Sex
Age
Variant
Histology
Case 1 Female 30 Unicentric Hyaline vascular Case 2 Female 57 Multicentric Plasma cell Case 3 Male 56 Multicentric Plasma cell
VIH HHV8 – – –
– + +
Treatment Surgery CHT, Steroids, Antivirals CHT, Steroids, Antivirals, Specific antibodies
Figure 1. Variant hyaline vascular
Discussion & conclusion: Castleman’s disease is an unusual pathology to be considered in patients with increased lymph nodes. Three hystological variants as well as its association with HHV8 and Kaposi’s sarcoma. Keywords: Castleman, Kaposi, HHV8.
Eduardo Santos Ribeiro, Dulcídia Sá, Zoya Klitna, Sandra Rodrigues, Rosa Jorge. Hospital Infante D. Pedro, E.p.e. - Aveiro, Portugal - Serviço De Medicina Ii Erythema nodosum (EN) is characterized by red or violet subcutaneous nodules that usually develop in a pretibial location and usually is associated with infections, drugs, sarcoidosis, collagen diseases, inflammatory bowel disease and other. The authors present a 40 years old patient, with history of alcohol abuse till about 1 year ago, pre-medicated with thiapride, oxazepam and oral contraceptive that came into the Emergency Room because of fever (37.9°C), pitting edema of the legs and erythematous eruptions since 3 weeks. On physical examination, the patient was afebrile, presenting with pitting edema of the legs and with bilateral, painful, erythematous, maculopapular eruptions, suggestive of erythema nodosum, without other relevant changes on physical exam. The laboratory showed ESR 45 mm (<25), CRP 11.78 mg/dL (<0.5), ADA 29.3 IU/L (4.8 – 23.1), SACE 84.3 IU/L (<40), negative ANA, ENA and ANCA, negative HIV, HCV and HBV serologies, negative ASO titer and blood culture. The Mantoux test and the Koch’s bacillus culture were also negative. CT scan showed multiple mediastinal adenopathies whose histologic examination made the diagnosis of sarcoidosis. Then was performed a fiberoptic bronchoscopy and bronchoalveolar lavage that showed a CD4/CD8 ratio of 5. The DLCO was normal. After 1 year of follow-up the patient has no symptoms and the SACE decreased to 45 IU/L (<40), without any therapy. Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that affects individuals worldwide and is characterized pathologically by the presence of noncaseating granulomas in involved organs. The thorax is involved in up to 90% but it can also affects the skin, eyes, heart, CNS, bones and joints. The prognosis of sarcoidosis is generally favourable. Complete spontaneous remission is seen in 60-80% and treatment should be given only to patients with maintained symptomatology, progressive lung disease, eye, CNS or heart involvement and malignant hypercalcemia.
P0273 MESENTERIC TUMOR. DIFFERENTIAL DIAGNOSIS AND MEDICAL TREATMENT OF SCLEROSING MESENTERITIS-MESENTERIC PANNICULITIS
Jose Manuel Rodriguez-Chinesta 1 , Fernando Cibrian 2 , Fernando Garcia-Ruiz 1 , Milagros Garcia 1 , Juan Diez 1 . 1 Usp Clinica La Esperanza. Vitoria-gasteiz; 2 Residencia Arana. Diputación De Alava Introduction: Sclerosing mesenteritis-mesenteric panniculitis is a chronic inflammatory unspecific process which involves mesentery, and its diagnosis must be performed by histology. It is a quite rare condition of unknown etiology. We report a new case and make a differential diagnosis with other inflammatory and tumoral diseases which affect to mesentery, epiplon and bowel. Case report: Woman of 25 with nor previous diseases of interest neither toxic habits. During the three months prior to admission at the hospital she began to feel a non-acute abdominal pain on the left hypochondrias, constipation and sometimes an acute abdominal pain after eating. The physical examination showed just a pain in the mesogastrio and left hypochondrias. A blood sample for a general analysis and serologies was normal. No tumoral markers were found. A chest X-ray and an intestinal transit were normal A mesenteric tumour of 7.5×6.2×6 cm. appeared in a CT and MNR scan. The tumour displaced stomach, pancreas and transverse colon. The mass had not calcium spitting inside. No regional adenopaties were found. After the discovering of the tumour the patient went into a laparotomy Then a mesenteric tumour could be seen which infiltrated stomach, pancreatic itsmo, transverse colon, mesocolon and small bowel mesentery. The mass included the superior mesenteric artery and vein. The microscopic exam with inmunohistochemist and PAS techniques was suitable for a sclerosing mesenteritis. No malignancy was observed. There was a marked inflammatory infiltrate. The patient received metilprednisolone bolus during three days followed by oral prednisone and colchicine. The symptoms disappeared four days after the beginning of treatment. Discussion & conclusion: This case raises the differential diagnosis with
S95
desmoids fibromatosis which has not the inflammatory component. We can also make a differential diagnosis with inflammatory pseudotumour actine-positive, and with inflamatory liposarcoma which is (as in our case) positive for S100 proteine but has atipic and multinucleous cells. Besides the liposarcomais a good definited mass which can be easily removed.
P0274 CASTLEMAN DISEASE: A CLINICAL AND HYSTOLOGICAL STUDY OF THREE CASES
Maria Fernandez Cardona, Margarita Ganzo Pion, Belen Macias Bou, Itxasne Cabezon Estevanez, Cristina Diez Romero, Paloma Diez Romero, Alejandro Fernandez Simon, Isabel Perez Tamayo, Miguel Angel Perez Tascon, Francisco Javier Torres Segovia. H.g.u.gregorio Marañon, Medicina Interna 3 Introduction: Castleman’s disease is a lymphoproliferative disorder associated with HIV, HHV8 and a number malignancies including Kaposi’s sarcoma and lymphoma. It can be unicentric (lymph nodes localized in head-neck and thorax) or multicentric (disseminated lymph nodes, hepatosplenomegaly, constitutional symptoms and laboratory abnormalities). There have been described three hystological variants: hyaline vascular, plasma cell and plasmablastic variant. A wide broad of therapies have been tried, including surgery, radiation, steroids, antiviral agent, specific antibodies, agents that inhibit cytokine activity, as well as chemotherapy. Objectives: The purpose of this study is to show the clinical presentation of an unsual hematological pathology and its therapeutical management. Materials & methods: In this descriptive study we recruited the pacients that presented pathological adenopaties at the admission in our hospital between January 2003 and December 2008. Results: A total of 595 patients diagnosed of pathological adenopaties were studied, three of them were diagnosed of Castleman’s disease. Two cases presented multicentric variant. Two of them were associated with HHV8, one of them with Kaposi’s sarcoma. Case 1
Case 2
Case 3
+ – – – – – + +
+ + + + + + + +
+ + + + + + + +
Lymph nodes Hepatoesplenomegaly Fever Weight loss Hypoalbuminemia Pancytopenia Hypergammaglobulinemia Increased erytrocythe sedimentation rate Sex
Age
Variant
Histology
Case 1 Female 30 Unicentric Hyaline vascular Case 2 Female 57 Multicentric Plasma cell Case 3 Male 56 Multicentric Plasma cell
VIH HHV8 – – –
– + +
Treatment Surgery CHT, Steroids, Antivirals CHT, Steroids, Antivirals, Specific antibodies
Figure 1. Variant hyaline vascular
Discussion & conclusion: Castleman’s disease is an unusual pathology to be considered in patients with increased lymph nodes. Three hystological variants as well as its association with HHV8 and Kaposi’s sarcoma. Keywords: Castleman, Kaposi, HHV8.