Hodgkin Disease & Malignant Lymphomas

66 HODGKIN’S DISEASE AND MALIGNANT LYMPHOMAS
Christina A. Finlayson M.D.

1. What is the differential diagnosis of lymphadenopathy?

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The significance of cervical, axillary, or inguinal lymphadenopathy depends on the characteristics of the lymph nodes and associated symptoms. Infection, autoimmune disease, and malignancy are all included in the differential diagnosis.

2. What historical information helps to direct the diagnostic investigation of lymphadenopathy?

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It is unusual for a patient older than 40 years to have nonspecific lymphadenopathy; over 70% of enlarged cervical lymph nodes in this age group are malignant. Patients younger than 40 years are more likely to have a nonspecific or infectious cause, although the mean age of Hodgkin’s lymphoma diagnosis is 32 years.
The duration of adenopathy helps with the diagnosis. A newly enlarged lymph node is more suggestive of infection, although an enlarging lymph node can undergo internal hemorrhage with a rapid increase in size. Travel and occupation history, exposure to pets, geographic area of residence, and sexual history provide clues to infectious agents. A history of smoking is associated with lung, upper gastrointestinal, and head and neck malignancy.
Systemic symptoms, including fever, weight loss, night sweats, and pruritus, are present in 30% of patients with Hodgkin’s and 10% of patients with non-Hodgkin’s lymphoma.

3. A 25-year-old man presents for evaluation of a 1-cm, soft inguinal lymph node that has been present for 1 month. How should the diagnostic evaluation proceed?

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Examine all draining lymph node basins including cervical, submandibular, auricular, occipital, supraclavicular, axillary, epitrochlear, inguinal, and popliteal. Supraclavicular adenopathy is virtually always associated with malignant or granulomatous disease. Peripheral adenopathy in the groin and axilla is often a response to trauma, frequently occult. The limb should be examined thoroughly.
A lymph node < 1 cm in size is usually not significant. Nodes > 2 cm are often malignant or granulomatous. Whereas hard nodes are typical of metastatic malignancy, soft nodes could be anything.
Reexamine the patient in 1 month. If regression has not occurred, biopsy the node.

4. A 48-year-old woman presents with a 3-cm, firm lymph node in the left supraclavicular area. How may her evaluation differ from that of the previous patient?

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The age of the patient, the size and consistency of the lymph node, and its location virtually mandate fine-needle aspiration (FNA) or open biopsy. Malignancy must be excluded. Hodgkin’s and non-Hodgkin’s lymphomas as well as metastatic disease from a primary intraabdominal, genital, lung, or breast primary frequently present this way. Head and neck tumors rarely metastasize to this location but tend to spread first to cervical lymph nodes.
A period of observation is not appropriate.

5. Should antibiotics be used during a “watch-and-wait” period when a specific site of infection has not been identified?

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Lymph nodes are rarely the target of invading organisms. When infected nodes are present, other signs of inflammation, including warmth, erythema, and pain, accompany the swelling. If a specific infection is not identified, the empiric use of “shot-in-the-dark” antibiotics does not have therapeutic or diagnostic benefit.

6. Can FNA be used if lymphoma is in the differential diagnosis?

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FNA is an established diagnostic tool used in the evaluation of breast, thyroid, and metastatic disease. When a patient presents with lymphadenopathy, often a diagnosis can be established from a lymph node aspirate that implicates a cancer other than lymphoma.
Establishing a definitive diagnosis of lymphoma by FNA is not reliable. The pathologist often requires intact lymph node architecture to reach a diagnosis and to provide accurate tumor typing. The recent addition of flow cytometry evaluation of the aspirate sample has increased the diagnostic yield of FNA for lymphoma. However, many aspirates appear normal and cannot be processed by flow cytometry. In this setting, most patients require surgical biopsy to obtain adequate tissue for histology as well as immunohistochemical evaluation.

7. How should a surgeon do a lymph node biopsy for suspected lymphoma?

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The primary role of the surgeon in lymphoma is to diagnose and stage the disease. The cervical lymph nodes are usually the site of involvement (75%), followed by the axillary (15%) and inguinal lymph nodes (9%). It is common for a primary node involved with a tumor to be accompanied by smaller reactive lymph nodes. Therefore, it is important to select the largest, most suspicious lymph node for biopsy. Because the architecture of the lymph node is important for the pathologist, the node should be removed in one piece. Do not crush, clamp, or cauterize the node. The node must go to the laboratory fresh, wrapped in saline-soaked gauze. Soaking the node in water or formalin distorts the cellular architecture.
Talk to your pathologist. A frozen section can determine whether you have enough tissue.

8. What are the clinical differences between Hodgkin’s and non-Hodgkin’s lymphoma?

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Hodgkin’s lymphoma usually presents with either a neck or a mediastinal mass. It arises first in the lymph nodes and rarely involves extranodal sites initially. It tends to spread contiguously to adjacent nodal stations rather than “skipping” to distant sites. Most patients present with early stage I or II disease. Epitrochlear, popliteal, or mesenteric nodal involvement is unusual. There is a bimodal age distribution with an early peak in the 20s and a later peak in the 60s.
Non-Hodgkin’s lymphomas originate from lymphocytes and also are called lymphocytic lymphomas. The incidence of these tumors has increased over the past 20 years. Some of this increase has occurred because of an association with AIDS, but this is not the whole story.
In contrast to Hodgkin’s lymphoma, non-Hodgkin’s lymphomas are often extranodal and spread noncontiguously. They rarely present as localized disease; bone marrow and liver involvement is common. Non-Hodgkin’s lymphoma involves epitrochlear, popliteal, and mesenteric lymph nodes as well as Waldeyer’s ring. It accounts for almost all gastrointestinal lymphomas. Most patients present with advanced-stage disease.

9. What is Waldeyer’s ring?

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This is the mucosa of the posterior oropharynx covering a bed of lymphatic tissue that aggregates to form the palatine, lingual, pharyngeal, and tubal tonsils. These structures form a ring around the pharyngeal wall. This may be the site of primary or metastatic tumor.

10. Why are tumors staged?

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Quantifying the size of the tumor, the presence of nodal disease, and presence or absence of distant metastasis (TNM staging) provides an estimate of tumor burden. Because the primary tumor burden of lymphoma arises in nodal tissue, the usual TNM staging system is not used. Measuring the extent of disease helps to determine therapy and to predict prognosis.
KEY POINTS: HODGKIN’S DISEASE

1. The primary role of the surgeon is to diagnose and stage the disease.
2. The cervical lymph nodes are the usual site of involvement (75%), followed by the axillary (15%) and inguinal (9%) lymph nodes.
3. The node should be removed in one piece and not crushed, clamped, or cauterized.
4. The node should go to the pathologist fresh wrapped in saline-soaked gauze.

11. How is lymphoma staged?

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History and physical examination elicit systemic symptoms and identify involved lymph node stations. Obtain a complete blood count, creatinine, liver function tests, erythrocyte sedimentation rate, lactate dehydrogenase, and alkaline phosphatase. If a chest radiograph is abnormal, a computed tomography (CT) scan of the chest is required. A computed tomography (CT) scan of the abdomen and pelvis and bilateral bone marrow aspiration and biopsy are required in all cases. Lymphangiography and staging laparotomy are controversial.

12. What staging system is used for Hodgkin’s and low-grade non-Hodgkin’s lymphomas?

Because lymphoma is a malignancy of the lymph nodes and the initial site of disease is rarely identifiable, the TNM staging system does not apply. Staging, therefore, is based on the distribution of the disease and systemic symptoms. Hodgkin’s and low-grade non-Hodgkin’s lymphomas use the Ann Arbor Staging Classification:
The subscript E denotes extralymphatic organ involvement, and the subscript S denotes splenic involvement in stage III or IV disease. E and S may be combined with involvement of both an extralymphatic site and the spleen.
Each stage is subdivided into either A or B. Patients without systemic symptoms are As. Patients with weight loss of more than 10% in the preceding 6 months, unexplained temperatures > 38°C, or drenching night sweats are classified as Bs. Pruritus is often included in the description of B symptoms but does not qualify for B classification when it is the only presenting systemic symptom.
For example, a 24-year-old man who presents with an asymptomatic mass in the neck, no systemic symptoms, and no other sites of disease on staging is classified as stage IA. A 70-year-old woman who presents with a localized small bowel lymphoma (low-grade) that involves the mesenteric (regional) lymph nodes and has had a temperature of 38.5°C over the past 6 weeks is classified as stage IIEB.

13. What staging system is used for intermediate- and high-grade non-Hodgkin’s lymphomas?

The National Cancer Institute Modified Staging System is used: Poor prognostic features include Karnofsky performance status < 70, B symptoms, any mass > 10 cm in diameter, serum lactate dehydrogenase > 500, or ≥ 3 extranodal sites of disease.

14. What is Karnofsky performance status?

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This is a scale used to quantify a patient’s activity level, which reflects the impact of the disease. If a patient has a disease but it does not interfere with activities, the performance status is 100%. As disease progresses, activity decreases and performance status falls. A bed-bound patient is at 10%.

15. What is the difference between clinical and pathologic staging?

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Clinical staging is based on history, physical examination, and radiographic evaluation. Abnormal lymph nodes identified by abdominal CT scan or lymphangiography imply clinical subdiaphragmatic disease. Pathologic staging requires the histologic conformation of all potentially involved tissues. Pathologic staging of abnormal lymph nodes identified by abdominal CT scan or lymphangiography requires staging laparotomy with biopsies. To identify the method of staging, a lower-case c for clinical staging or p for pathologic staging precedes the staging nomenclature. For example, cIII indicates a tumor staged clinically with abnormal lymph nodes identified by abdominal CT scan or lymphangiography. If a staging laparotomy is performed and pathologic confirmation identifies involved lymph nodes, the tumor is stage pIII.

16. What is a staging laparotomy?

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This is a midline incision that permits attention to lymph node-bearing areas. Splenectomy is performed first, followed by wedge and core biopsies of each lobe of the liver. Lymph nodes are obtained from the celiac, mesenteric, portal, paraaortic, and paracaval areas. In premenopausal women, an oophoropexy secures the ovaries behind the uterus and preserves fertility in approximately 50% of women who require pelvic radiation. When the abdomen is closed, bone marrow biopsies are performed bilaterally.

17. What are the indications for staging laparotomy?

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A staging laparotomy should be performed only when the results may change the clinical stage and when a change in stage will alter the planned treatment. Pathologic staging of surgically removed tissue is more accurate than clinical staging. In the Stanford experience, 43% of patients had a change in stage after laparotomy. Approximately 30% of patients in clinical stages (CS) I and II are “upstaged” to pathologic stage III or IV disease after surgery. Conversely, 20% of patients with clinical stage III or IV disease are “downstaged.” In some subgroups of patients, however, the risk of subdiaphragmatic disease is so low (< 10%) that staging laparotomy rarely adds information. These subgroups include all CSIA women and CSIA men with a high neck presentation, lymphocyte-predominant histology, or mediastinal-only disease.
Obscuring the role of staging laparotomy in treating Hodgkin's disease is its lack of effect on survival because of the highly effective salvage chemotherapy available for patients who relapse. Evidence indicates, however, that patients staged surgically have a lower incidence of recurrence and, therefore, are less likely to require a second course of treatment.
Staging laparotomy is not performed for non-Hodgkin's lymphoma.

18. How is Hodgkin’s lymphoma treated?

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Stage I and IIA disease may be treated with radiation alone if the tumor is not bulky and the prognostic factors are favorable. More advanced disease requires adjuvant chemotherapy. Combinations include three or more of the following: mechlorethamine, vincristine, procarbazine, prednisone (MOPP), doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD).

19. What is the Working Formulation for non-Hodgkin’s lymphoma?

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Non-Hodgkin’s lymphoma includes many diverse histologic patterns, each with its own natural history and prognosis. Early attempts to classify these subtypes resulted in six chaotic classification schemes. The Working Formulation was created to standardize the nomenclature for non-Hodgkin’s lymphomas. It categorizes each cytologic description into three general categories: low, immediate, and high grade. Each category has a similar natural history, treatment plan, and prognosis.

20. Does the natural history for each category of non-Hodgkin’s lymphoma differ?

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Yes. Low histologic grades grow slowly, with waxing and waning symptoms over a long period. High histologic grades progress rapidly and, if left untreated, are fatal in a short period. Ironically, chemotherapy has been most successful in the intermediate and aggressive subtypes. Intermediate-grade lymphomas often respond to standard combination chemotherapy. Aggressive lymphoma, when treated promptly with combination chemotherapy, has a 75% complete response rate and 50% chance of long-term survival.

21. Is there a role for the surgical treatment of lymphoma?

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Yes. Localized non-Hodgkin’s lymphoma of the gastrointestinal (GI) tract most commonly arises from the stomach. It originates in the lymphoid tissue of the submucosa. Surgery has been the mainstay of treatment, and complete resection of early-stage disease is frequently curative. Patients with more advanced disease may benefit from adjuvant radiation and chemotherapy.
Lymphoma at other locations within the GI tract often presents as a surgical emergency. The diagnosis is often made at the time of the operation, and attention is focused on treating perforation, obstruction, or hemorrhage. Resection of the tumor is indicated if the disease appears localized.

22. What is the risk of a second cancer in patients successfully treated for Hodgkin’s lymphoma?

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It is higher. The more common of these second cancers are lung, breast, sarcoma, leukemia, and non-Hodgkin’s lymphoma. Patients who received radiation therapy to the cervical area require annual thyroid function testing to detect radiation-induced hypothyroidism. Young women who received thoracic radiation require screening for breast cancer. Annual mammography should be started 10 years after treatment but no later than age 40 years.

References
WEB SITES

1. http://www.lymphoma.org
2. www.cancer.gov
3. www.cancer.org

BIBLIOGRAPHY
1. Bazemore AW, Smucker DR: Lymphadenopathy and malignancy. Am Fam Physician 66:2103-2110, 2002. Medline Similar articles
2. Fleming I (ed): The Surgeon and Malignant Lymphoma. Surgical Oncology Clinics of North America. Philadelphia, W.B. Saunders, 1993.
3. Hoppe RT: NCCN practice guidelines for Hodgkin’s disease. Oncology 13:18-25, 1999.
4. Nynadoto P, Muhonen T, Joensu H: Second cancer among long-term survivors from Hodgkin’s disease. Int J Radiat Oncol Biol Phys 42:373-378, 1998.
5. Patient Care Committee of the Society for Surgery of the Alimentary Tract (SSAT): Splenectomy. J Gastrointest Surg 3:218-219, 1999.
6. Walsh RM, Heniford BT: Role of laparoscopy for Hodgkin’s and non-Hodgkin’s lymphoma. Semin Surg Oncol 16:284-292, 1999. Medline Similar articles Full article
7. Young NA, Al-Saleem TI, Ehya H, Smith MR: Utilization of fine-needle aspiration cytology and flow cytometry in the diagnosis and subclassification of primary and recurrent lymphoma. Cancer 84:252-261, 1998. Medline Similar articles Full article
8. Zelenetz AD, Hoppe RT: NCCN: Non-Hodgkin’s lymphoma. Cancer Contrl 8(suppl 2):102-113, 2001.

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