Sarcoidosis and Multiple Myeloma: A Case Report and Literature Review.

The existence of a sarcoidosis-lymphoma syndrome has been previously proposed since the relation between sarcoidosis and an increased risk of lymphoproliferative disorders is well established. Multiple myeloma is a malignant multifocal proliferation of clonal plasma cells within the bone marrow, and its association with sarcoidosis has been rarely described. We present a concurrent diagnosis of sarcoidosis and multiple myeloma and make a brief analysis of the reported cases in the literature. A 65-year-old woman underwent surgery for the excision of a wrist mass that presented 3 years before. Histological analysis showed sarcoid-type epithelioid granulomas without necrosis, establishing soft tissue sarcoidosis. Further evaluation revealed marked interstitial lung parenchyma lesions and large intrathoracic adenopathies. Bronchofibroscopy with transbronchial biopsy confirmed lung sarcoidosis. In addition, blood analysis showed monoclonal IgG kappa gammopathy. A bone marrow biopsy confirmed hypercellularity with 60% plasma cells and plasmocyte infiltration. Thus, the diagnosis of systemic sarcoidosis and multiple myeloma was established simultaneously. In a brief review of the literature, we identified 33 reports of cases with both sarcoidosis and multiple myeloma. We point out the importance of a high level of suspicion for the association of sarcoidosis with malignant haematological diseases such as multiple myeloma.

[Oncocytic tumors of the adrenal gland]. / Tumores oncocíticos da supra-renal.

The oncocytic tumors of the adrenal gland are rare. To date there's only 147 cases published. The authors present a case of a 34 year-old man admitted to the hospital with lumbar pain and fever. At medical examination a mass was found in the left upper quadrant of the abdomen. The hormonal measurements of the adrenal gland were normal and the abdominal angio CT showed a left retroperitoneal lesion measuring 145 x 157 x 128 mm with extensive necrotic and hemorrhagic areas. The patient underwent a complete surgical resection of the lesion. The mass weighted 1495g and the histological exam revealed an oncocytic tumor of the adrenal gland with uncertain potential. Most of the oncocytic tumors are non functioning and must be considered in the differential diagnosis of adrenocortical tumors. The biologic behavior and the prognosis of these tumors are variable and still need a better definition. Due to the rarity of these tumors the authors made a revision on the published bibliography.

Os tumores oncocíticos da supra-renal são neoplasias pouco frequentes. Atualmente existem 147 casos publicados. Os autores descrevem o caso de um homem de 34 anos admitido no hospital por lombalgias e febre. À observação identificava-se massa no quadrante superior esquerdo do abdomen. Os doseamentos das hormonas da supra-renal foram normais e a angiotomografia abdominal mostrou lesão retroperitoneal esquerda com 145 x 157 x 128 mm com extensas áreas necrosadas e hemorrágicas. O doente foi submetido a ressecção cirúrgica completa da lesão. O tumor pesava 1495 g e o exame anatomo-patológico concluiu tratar-se de tumor oncocítico da supra-renal com potencial maligno incerto. A maioria dos tumores oncocíticos é não funcionante e devem ser considerados no diagnóstico diferencial de tumores do córtex da supra-renal. O comportamento biológico e o seu prognóstico são variáveis e ainda estão mal estabelecidos. A propósito da raridade deste tipo de tumores os autores fazem uma revisão da bibliografia publicada.

[Hypokalemic paralysis: the first presentation of primary Sjögren's syndrome]. / Paralisia Por Hipocaliémia: Manifestação Inaugural da Síndrome de Sjögren Primário.

The Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren's syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren's syndrome.