Staging

Table 15-9 outlines the modified Ann Arbor classification of HD, and Table 15-10 lists the investigations in HD.

Abdominal and pelvic CT are useful modalities to evaluate spread of the disease in the abdomen and pelvis including the retroperitoneum. The limitations of CT include:

• Difficulty in detecting splenic hilar, mesenteric, celiac, or porta hepatis lymph node disease, and because children, compared to adults, have little or no retroperitoneal fat there may be difficulty recognizing retroperitoneal lymph nodes.

Table 15-9. Modified Ann Arbor Classification of Hodgkin Disease

Stage Description

I Involvement of a single lymph node region (I) or of a single extralymphatic organ or site (IE) by direct extension

II Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of an extralymphatic organ or site and of one or more lymph node regions on the same side of the diaphragm (IIE); optionally, the number of node regions involved is indicated by a subscript

1111 Involvement of lymph node regions on both sides of the diaphragm (III);

may or may not be accompanied by localized involvement of an extralymphatic organ or site (IIISE); abdominal disease is limited to the upper abdomen: spleen, splenic hilar node, celiac node, porta hepatis node

1112 Involvement of lymph node regions on both sides of the diaphragm;

abdominal disease includes para-aortic, mesenteric, iliac, or inguinal lymph node involvement with or without disease in the upper abdomen

1113 Pelvic node involvement with or without any other abdominal disease

IV Diffuse or disseminated involvement of one or more extralymphatic organs or tissues with or without associated lymph node enlargement; extralymphatic organs are defined as those other than lymph nodes, spleen, thymus, Waldeyer ring, appendix, and Peyer patches; liver or bone marrow involvement always indicates stage IV disease; biopsy—document involvement of stage IV sites is also denoted by letter suffixes: marrow, M+; lung, L+; liver, H+; pleura, P+; bone, O+; skin and subcutaneous tissue, D+

Subclassification

A: Denotes no specific symptoms

B: Denotes specific symptoms as follows:

1. Unexplained body weight loss of more than 10% over a 6-month period

2. Unexplained recurrent fever with temperature above 38°C

3. Night sweats

From Carbone PP, Kaplan HS, Musshoff K, et al. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res 1971;31:1860, with permission.

Table 15-10. Investigations in Hodgkin Disease

History: fever, night sweats, weight loss, pruritus

Physical examination: peripheral nodes, liver, spleen, bone tenderness, Waldeyer ring Hematologic studies: Complete blood count Erythrocyte sedimentation rate Biochemical studies:

Liver function tests (serum glutamic-oxaloacetic transaminase, serum glutamic pyruvic transaminase, alkaline phosphatase, total protein/albumin, lactic dehydrogenase, bilirubin)

Renal function studies (blood urea nitrogen, creatinine, serum electrolytes, urinalysis) Serum copper Fibrinogen Haptoglobin Immunoglobulins Serum ferritin and transferrin Serum P2-macroglobulin Serum-soluble interleukin-2 receptor (sIL-2R) T4/TSH LH/FSH Immunologic evaluation:

Absolute lymphocyte count T- and B-cell counts T-cell function studies Anthropomorphic measurements and Tanner staging Radiologic studies:

Chest radiograph (posterior, anterior, and lateral)a

CT scan—neck for soft tissue (in presence of high cervical node to evaluate Waldeyer ring involvement)

CT scan—chest (with intravenous contrast) (special attention to thoracic inlet, axillae, and crura of diaphragm) CT of abdomen and pelvis (with oral and intravenous contrast) MRI of bones (if bone marrow or bony involvement suspected)

CT or MRI of spine (if bony tenderness or symptoms of cord compression suspected) Bone scan (optional)

Gallium scan (for all patients and specifically the cases with mediastinal mass) FDG-PET scan

Ultrasound examination of abdomen (special attention to liver and spleen) Exploratory laparotomy for surgical staging.6 Rarely, if ever, required.

Multiple lymph node biopsies—para-aortic, mesenteric, celiac, porta hepatis, pancreatic, splenic hilar, iliac Splenectomy

Liver biopsy—needle and wedge (from both lobes) Bone marrow biopsy (minimum two biopsies from each side of iliac bones) Midline transposition of ovaries and oophoropexy (out of radiation port) in girls and young women if there is a possibility of future pelvic irradiation

"Radiograph should be taken with maximal inspiration in the upright position at a source-skin distance of 2 m.

6Bone marrow needle biopsy (from multiple sites, i.e., anterior and posterior both iliac bones) and percutaneous liver biopsy should be performed prior to surgical staging in patients suspected of having stage IV disease.

• CT reveals splenic disease, with only a 19% sensitivity. However, when the splenic index (SI) is utilized, accurate determination of splenic HD is about 59%. FDG-PET scans can identify splenic HD with 92% accuracy.

• MRI has limited value and is only useful in identifying nodes from loops of bowel when doubt exists on CT.

Radionuclide scanning with 67Ga citrate provides true-positive data in only 40% of patients, with false-negative rates of 60% in patients, thus limiting its usefulness for assessing the subdiaphragmatic area in children.

Staging laparotomy is a major surgical procedure. Potential complications include wound infection, retroperitoneal hematoma, subphrenic abscess, pancreatitis, and acute pulmonary complications (e.g., atelectasis and pneumonia). Late complications such as adhesions, leading to intestinal obstruction, may occur even in those patients who do not receive abdominal radiation. The incidence is 3-12% in children.

When radiation alone is used, surgical staging is helpful to define subdiaphragmatic disease in anticipation of abdominopelvic radiation. When chemotherapy is used, either alone or in combination with radiation (which is the preferred mode of treatment of HD in most cases), the current trend is to rely on clinical staging because it has been shown that effective chemotherapy is sufficient to eradicate occult micro-foci of subdiaphragmatic disease.

Surgical staging is no longer necessary because of the following:

• Increased use of systemic therapy in children.

• Advances in diagnostic imaging technology, which permit more accurate evaluation of retroperitoneal lymph nodes.

• Recognition of life-threatening sequelae, including overwhelming bacterial infections in asplenic children. Overwhelming sepsis with encapsulated bacteria occurs in 1-2% of patients with HD who have undergone splenectomy. The management of the splenectomized patient is described in Chapter 26.

For these reasons, surgical staging is rarely utilized today and in an ongoing Children's Oncology Group (COG) study, surgical staging is not recommended.

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