6.6.2.4 Other Locations

•Central nervous system (CNS), cranial and peripheral nerves, skin, muscles, bone, thorax, gonads, orbit, parotid gland, epidural region

•Symptoms depend on location

6.7Differential Diagnosis Among the Different Forms of NHL (In Ranking of Frequency)

•Burkitt Lymphoma (BL), sporadic form:

–Abdomen (25%) with ascites and pleural effusion

–Pharyngeal and retropharyngeal area, including sinuses

–Bone and bone marrow involvement, 20–40%

–Involvement of CNS is rare

•Burkitt Lymphoma (BL), endemic form:

–Mostly in Equatorial Africa

–Jaw area in about 70% of children less than 5 years of age and in 25% of children more than 14 years of age

–Abdomen

–Bone marrow frequency of involvement, 8%

–CNS and cranial and peripheral nerves including epidural involvement: more frequent in the sporadic than the endemic form

•Burkitt like Lymphoma (BLL):

–Children older than those with endemic BL

–In contrast to endemic BL, more frequent involvement of liver, spleen, and mediastinum

•Lymphoblastic lymphoma (LL):

–Intrathoracic and mediastinal in 50–70% of patients

–Lymphadenopathy in 50–80% of patients, especially supradiaphragmatic

–Differential diagnosis: ALL has more than 25% of blasts in the bone marrow analyses at diagnosis

•Anaplastic large-cell lymphoma (ALCL):

–Slow progression

–Involvement of lymph nodes, skin, bone, mediastinum, liver, and spleen

6.7.1Differential Diagnosis of Other Disorders

•Lymph node enlargement in infectious diseases

•Autoimmune lymphoproliferative syndrome (ALPS)

•Hodgkin disease

•Metastatic disease of sarcomas or neuroblastomas

•ALL:

–By definition, bone marrow analysis with more than 25% of blasts; with less than 25% of blasts in bone marrow, NHL stage IV

–Overlap between T-cell ALL and T-cell NHL is possible