Author(s)

Tammo R, Mangov A, Takoeva T, Khomenko T

Patient

male, 4 year(s)

A 4-year-old male patient presented with progressive dyspnea, which he had had over more than a month, cyanosis, general weakness, decreased appetite, low grade fever, night sweats, and weight loss.

A 4-year-old male patient presented with progressive dyspnea, which he had had over more than a month, cyanosis, general weakness, decreased appetite, low grade fever, night sweats, and weight loss. A medical examination that was done, revealed the enlargement of the cervical lymph nodes. Laboratory analysis results revealed that the patient had anemia and leukocytes. A contrast-enhanced CT done showed a large subcarinal tumor mass extending downwards to the diaphragm, other pericardiac tumor masses were noted in the anterior mediastinum with a dorsal displacement of the heart. The parasternal soft tissues were involved by the direct extension from anterior mediastinal nodes. The tumor mass was seen on the left paravertebral area. A left pleural mass was seen with infiltration of the chest wall. It was noted that pleural effusion caused compression of the left lung and left pulmonary consolidation with an air bronchogram. The CT showed mediastinal, abdominal and retroperitoneal lymphadenopathy. Multiple low-attenuation lesions were seen within the liver and the spleen; there were also multiple, slightly hypointense lesions in the left kidney. The diagnosis of ascites was confirmed on doing a lymph nodal biopsy.

Although Hodgkin’s lymphoma is known to be the most common mediastinal lymphoma, it accounts for only about 20%–30% of all lymphomas, that is, 1% of all malignancies [1]. Four histologic subtypes are described in the Rye classification system: nodular sclerosing (75%–80%), lymphocyte predominant (10%), mixed cellularity (10%), and lymphocyte depleted (5%). Pathologically, the diagnosis of a Hodgkin’s lymphoma is established by the identification of Reed-Sternberg cells. Patients with a Hodgkin’s lymphoma tend to present with a painless lymph node swelling, often in the upper body area but it can affect almost any node or related lymph system organ, general fatigue, weight loss—usually at least 10% over a short time, fever which can come and go, night sweats, often drenching itching skin. Patients with mediastinal disease tend to be of a younger age group than those without mediastinal disease [2]. Although up to 85% of patients with Hodgkin's lymphoma have intrathoracic disease, isolated mediastinal disease is found to be uncommon (10%) and there is typically adenopathy present elsewhere. Only 15% of patients with intrathoracic Hodgkin’s lymphoma have enlargement of a single lymph node group and only rarely are the posterior or paracardiac lymph node groups involved. Pulmonary parenchymal involvement can be found in up to 12% of patients [2] and is almost invariably associated with hilar or mediastinal nodal disease. Pleural effusions are usually secondary to lymphatic or venous obstruction. Chest wall involvement usually occurs secondary to direct extension from bulky adenopathy or from diseased lung parenchyma [3]. Splenic involvement is typically diffuse, and only a small minority of cases manifest with nodules larger than 1 cm in diameter. Hodgkin's disease of the liver is almost invariably associated with disease of the spleen. Renal involvement is known to be extremely rare. Hodgkin's disease is staged according to the Ann Arbor staging system. Stage I: Involvement of one nodal site. Stage II: Involvement of more than one nodal site, limited to one side of the diaphragm. Stage III: More than one nodal site, both sides of the diaphragm. Stage IV: Metastatic disease. "S" indicates splenic involvement. "E" indicates local extension. CT has been evaluated for its role in the initial workup of patients with Hodgkin's disease; CT can detect intrathoracic sites of disease not detected on CXR in up to 20% of patients [4]. On CT, there is usually an asymmetric, anterior mediastinal soft tissue mass which may invade the chest wall. Bulky mediastinal nodal disease can be associated with an interstitial edema due to lymphatic or venous obstruction. On MRI, a lymphoma typically demonstrates a homogeneous low signal intensity similar to muscle as seen on T1 images, and a high or mixed signal as seen on T2 images. During treatment, the signal intensity changes [5]. An active, untreated tumor contains an excess of free water, which increases the signal intensity on T2 images [5]. On successful treatment, cellular elements and the water content of the tumor are reduced, while collagen and fibrotic stroma increase [4]. PET imaging with FDG has demonstrated a high sensitivity for the detection of abnormal lymph nodes in patients with lymphomas and for patient staging.