Staging

Staging of NHL requires an expeditious investigation to determine the clinical extent of the disease, the degree of organ impairment and biochemical disturbance present. Staging laparotomy to determine precise pathologic staging of the disease is not indicated.

Chemotherapy is the therapeutic mainstay because of the multicentric origin of NHL. Radiation therapy alone, with fields determined by an anatomic staging system, is not employed. Testicular involvement is present in less than 4% of males at diagnosis. Table 16-5 lists a staging system that enables a uniform interpretation of results of various treatment regimens. This classification recognizes the common occurrence of dissemination to either the bone marrow or the CNS, or both, and the fact that it is indicative of a poor prognosis.

Table 16-5. Staging System for Childhood Non-Hodgkin Lymphoma: Murphy Classification

Stage I

A single tumor (extranodal) or single anatomic area (nodal) with the exclusion of mediastinum or abdomen

Stage II

A single tumor (extranodal) with regional lymph node involvement Two or more nodal areas on the same side of the diaphragm

Two single (extranodal) tumors with or without regional lymph node involvement on the same side of the diaphragm A resectable primary gastrointestinal tract tumor, usually in the ileocecal area, with or without involvement of associated mesenteric nodes only"

Stage III

Two single tumors (extranodal) above and below the diaphragm Two or more nodal areas above and below the diaphragm All primary intrathoracic (mediastinal, pleural, thymic) tumors All extensive primary intra-abdominal disease"

All paraspinal or epidural tumors, regardless of other tumor site (or sites)

Stage IV

Any of the above with initial involvement of central nervous system or bone marrow6 or both

"A distinction is made between apparently localized GI tract lymphoma versus more extensive intra-abdom-inal disease because of their quite different pattern of survival after appropriate therapy. Stage II disease typically is limited to a segment of the gut plus or minus the associated mesenteric nodes only, and the primary tumor can be completely removed grossly by segmental excision. Stage III disease typically exhibits spread to para-aortic and retroperitoneal areas by implants and plaques in mesentery or peritoneum, or by direct infiltrations of structures adjacent to the primary tumor. Ascites may be present, and complete resection of all gross tumor is not possible.

6Bone marrow involvement is defined as greater than 5% and less than 25% replacement of marrow elements by tumor without circulating blast cells.

From Murphy SB: Current concepts in cancer: Childhood non-Hodgkin's lymphoma. N Engl J Med 299:1446, 1978, with permission.

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