Rare Presentation of Large Cell Lymphoma.

Lymphoma is a common cancer of childhood. Its pathologic subtype of anaplastic large cell Non-Hodgkins Lymphoma (NHL) is rare and spread to the central nervous system (CNS) is even rarer. We present here such a case who presented to us with an acute history of fever and diplopia and co-existent polyarthritis since 8 years diagnosed as NHL with CNS involvement. CNS involvement is a rare presenting manifestation of NHL and is an important cause of morbidity and mortality in these patients. Chronic arthritis could be a risk factor or manifestation of immunodeficiency or immune dysregulation when associated with lymphoma. It is important to suspect NHL in children presenting with these symptoms for prompt evaluation and better clinical outcome.

A case of inflammatory myofibroblastic tumor originated from the greater omentum in young adult / 대한외과학회지

Inflammatory myofibroblastic (IMF) tumor is a rare solid tumor that often affects children. IMF tumors occur primarily in the lung, but the tumor may affect any organ system with protean manifestations. A 22-year-old woman was evaluated for palpable low abdominal mass that had been increasing in size since two months prior. Abdominal computed tomography showed a lobulated, heterogeneous contrast enhancing soft tissue mass, 6.5 x 5.7 cm in size in the ileal mesentery. At surgery, the mass originated from the greater omentum laying in the pelvic cavity and was completely excised without tumor spillage. Histologically, the mass was a spindle cell lesion with severe atypism and some mitosis. Immunohistochemistry for anaplastic lymphoma kinase-1 revealed that the lesion was an IMF tumor. Because of its local invasiveness and its tendency to recur, this tumor can be confused with a soft tissue sarcoma. Increasing physician awareness of this entity should facilitate recognition of its clinical characteristics and laboratory findings.

Genetics in heritable pulmonary arterial hypertension / 대한내과학회지

Pulmonary arterial hypertension is caused by vascular remodeling including muscularization of arteries, loss of small precapillary arteries, and formation of neointima and plexiform lesion, resulting in a progressive increase in pulmonary vascular resistance. About 70% of heritable pulmonary arterial hypertension and 10% to 40% of idiopathic pulmonary arterial hypertension patients possess mutations in bone morphogenetic protein receptor, type 2 (BMPR2), which is a type II receptor of TGF-beta superfamily. Very rarely, mutations in another receptors of TGF-beta superfamily, activin-like kinase-type 1 (ALK1) and endoglin (ENG) are found in pulmonary arterial hypertension patients with hereditary hemorrhagic telangiectasia. Genetic screening is useful to identify family members who are mutation carriers in heritable pulmonary arterial hypertension families.

Linfoma anaplásico de células grandes ALK-1 positivo, primario de pulmón: Informe de un caso pediátrico / ALK-1 positive anaplastic large cell lymphoma of the lung: Report of a pediatric case

El linfoma no Hodgkin anaplásico de células grandes, positivo al antígeno Ki-1, es una entidad bien reconocida. La característica clínica usual es la linfadenopatía con extensión mediastinal y en la enfermedad extranodal, que ocurre en la mitad de los casos, es la piel el sitio más común, con afección poco frecuente a médula ósea, pulmón y sistema nervioso. La mayor frecuencia de presentación del linfoma anaplásico de células grandes es en la población joven, con pronóstico más favora ble que en la población adulta. El linfoma anaplásico de células grandes primario de pulmón es una entidad clínica rara. Su comportamiento clínico corresponde a un linfoma de alto grado y en la mayoría de los casos se presentan al diagnóstico como una enfermedad en estadio avanzado. Se informa un caso pediátrico de linfoma anaplásico de células grandes primario de pulmón, ALK 1 positivo, que es un marcador pronóstico y específico de esta entidad.

Ki 1 anaplastic large cell non Hodgkin lymphoma is a well recognized clinical entity. The main clinical feature includes lymphadenopathy with mediastinal sparing. In the extranodal disease, which occurs in approximately half of the cases, the skin is the most common site; bone marrow, lung and central nervous system are generally not involved. Anaplastic large cell lymphoma is more common among young people, in whom the prognosis is more favorable. Primary anaplastic large cell lymphoma of the lung is a rare clinical entity. Its clinical expression is similar to a high grade malignant lymphoma, and in most cases the diagnosis is made in advanced stages. We present a pediatric case with ALK1 positive anaplastic large cell lymphoma of the lung.