Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version.

TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia).

Brown adipose tissue demonstrating intense FDG uptake in a patient with mediastinal pheochromocytoma.

We present an interesting case with a central mediastinal pheochromocytoma showing intense F-18 fluorodeoxyglucose (FDG) uptake in tumor and systemic brown adipose tissue (BAT) mimicking metastases. The findings of hypertension and high plasma catecholamine concentration suggested the presence of pheochromocytoma. Mediastinal tumor showed intense FDG uptake and faint uptake of I-131 metaiodobenzylguanidine. Intense FDG uptake was demonstrated in cervical, paravertebral, mediastinal, and perirenal regions. Positron emission tomography and computed tomography (PET/CT) revealed uptake in a fat density area suggesting that the FDG uptake had occurred in BAT. The mediastinal tumor was resected along with an adhesion to the left atrial wall and pathologically confirmed as pheochromocytoma. The plasma catecholamine concentration and blood pressure then reverted to normal. The FDG uptake in BAT disappeared after tumor resection.