Late Recurrence of Hodgkin’s Disease After Partial Splenectomy By J.M. Slaiby, J.P. Crowley, and J.F. Amaral

Providence, Rhode Island

@The use of laparotomy and splenectomy for staging purposes in patients with Hodgkin’s disease (HD) gained popularity in the early 1970s.’ Accurate staging and more effective treatment regimens, including combined chemother- apy and irradiation, have resulted in improved patient sur- vival rates. Similarly, an increased number of late complica- tions have been reported, including the development of thyroid disease, second malignancies, and septic complica- tions related to splenectomy.2,3 Partial splenectomy has been proposed as one method of preventing overwhelming post- splenectomy sepsish6 The authors present a case of recur- rence of HD, which occurred in the splenic remnant 13 years after the initial treatment. This case demonstrates that the spleen is a potential for recurrent intraabdominal Hodgkin’s disease after partial splenectomy; thus, the use of partial splenectomy for HD should be discouraged. Copyright o 1996 by W.B. Saunders Company

INDEX WORDS: Partial splenectomy, Hodgkin’s disease.

T HE POSSIBILITY that after partial splenectomy the splenic remnant might be a site of recurrent

Hodgkin’s disease (HD) has been considered but not reported previously. Partial splenectomy, as part of a staging procedure for HD, represents a potential method for preservation of the immunologic function of the spleen and prevention of overwhelming post- splenectomy sepsis.

CASE REPORT

An 11-year-old girl initially presented with a a-month history of painful cervical lymphadenopathy. She reported no other symp- toms, had no medical illness previously, and had not had any surgical procedures.

The initial physical examination showed several freely mobile, tender cervical nodes, which measured between 1 and 1.5 cm in diameter. The remainder of the physical findings were unremark- able. A chest radiograph showed upper mediastinal enlargement. A supraclavicular lymph node biopsy was performed, and the histopathology was consistent with nodular sclerosing Hodgkin’s lymphoma. The results of an intravenous pyelogram, lymphangio- gram, and liver-spleen scan were normal.

She underwent a staging laparotomy and partial splenectomy, with removal of approximately one third of the spleen. Pathological examination of the excised segment of spleen showed no evidence of disease. Her case was staged as HD type IIa. Over a 4-week period she received 4.000 cGy of radiation, using a mantle technique to the paraaortic and splenic pedicle areas.

She was seen at our institution 13 years after completion of therapy for HD, complaining of severe left-upper-quadrant and epigastric abdominal pain. The physical examination showed left-upper-quadrant tenderness. The spleen was palpable 3 cm below the costal margin. Her initial hemoglobin level was 8.2 g/dL, and the white blood cell count was 11,700.

During laparotomy a large amount of blood was found in the peritoneal cavity. The spleen was replaced with an inhomogeneous tumor mass that was adherent to the lateral and posterior abdomi-

Journa/ofPed/afr/c Surgery, Vol31, No 5 (May), 1996: pp 731-732

nal wall as well as to the diaphragm. The spleen and a portion of the diaphragm were resected en bloc. A second mass (measuring 7 x 4 x 3.5 cm), adjacent to the superior pole of the left kidney, also was excised. Pathological examination of the specimens showed an enlarged spleen and retroperitoneal lymph nodes, which were massively involved with HD of the nodular sclerosing type. Liver biopsy specimens were negative for tumor. Clinically and pathologically, the case was stage III A Sl.

Postoperative bone marrow biopsy and chest CT results were negative. The patient completed six cycles of chemotherapy (Adria- mycin, 25 mg/m2; bleomycin, 10 U/m2; vinblastine, 6 mg/m2; DTIC, 375 mg/m2) and remains disease-free 48 months after completion of therapy for recurrence (18 years after the initial presentation).

DISCUSSION

Before the advent of effective therapy, less than 1% of patients with HD survived more than 10 years after the diagnosis was established. However, in re- cent years, relapse within 3 years after diagnosis has accounted for at least 25% of all recurrences.7

The use of laparotomy with splenectomy for staging HD gained popularity in the early 197Os, after the initial report from Stanford University Medical Cen- ter.2 Since then, others have reported that staging laparotomy has allowed more accurate and selective treatment regimens, with the final pathological stage being altered in 25% to 50% of patients.1,3 The spleen remains the most frequently involved intraabdominal organ and is the only site involved in 50% of cases with a positive laparotomy result1 Abdominal disease in the absence of splenic involvement is rarely found.

Partial splenectomy as part of a staging laparotomy has been proposed as an alternative to complete splenectomy, the rationale being that removal of half or more of the splenic tissue will preserve normal splenic immunologic function. Boles et al reported on 10 children who were staged for HD with partial splenectomy.6 All 10 had one third to one fourth of the spleen removed, and only one spleen had histologi- cal evidence of HD. During the average follow-up period of 17 months, there were no reported deaths, episodes of sepsis, or evidence of HD recurrence. The

From the Division of Hematology, Department of Medicine, and the Department of Surgery, Rhode Island Hospital, Brown University School of Medicine, Providence, RI.

Address reprint requests to J.M. Slaiby, MD, Department of Surgery, Rhode Island Hospital, APC 120, 593 Eddy St, Providence, RI 02903.

Copyright o 1996 by W B. Saunders Company 0022-3468/96/3105-0032$03.00/O

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patient described in the present report was one of the original cases reported by Boles et al.

In an attempt to analyze the risk of disease under- staging by partial splenectomy, Dearth et al4 studied the spleens of 112 patients staged with laparotomy. Of these, 13 (11.6%) demonstrated limited splenic involvement, with five or fewer nodules localized to a single pole of the spleen and with no visible subcapsu- lar involvement. These investigators concluded that partial splenectomy could not exclude occult splenic involvement. Similarly, Sterchi et aI5 reviewed the spleens of 180 patients, which had been removed for staging HD. They found a 6.2% risk of understaging with partial splenectomy, and suggested that this subset of patients would be consistently undertreated and thus have a poorer survival rate.

Partial splenectomy has been suggested as an alternative procedure for staging HD. The proposed benefits of this procedure are preservation of splenic immunologic function and reduction in the risk of postsplenectomy sepsis. The risks associated with partial splenectomy are twofold. As has been shown,4,5 there is the risk of underestimating the stage of disease and, in addition, an increased risk of recur- rence in areas such as the splenic remnant, which may be inadequately treated. This case shows that partial splenectomy may fail to eliminate a potential source of recurrent intraabdominal HD-the splenic rem- nant. The surgeon should be critical of partial splenec- tomy because patients who have had this procedure are at risk for the development of recurrent disease many years after completion of therapy.

REFERENCES

1. Taylor MA, Kaplan HS, Nelson TS: Staging laparotomy with splenectomy for Hodgkin’s disease: An alternative approach. World J Surg 9:449-460,1985

2. Glatstein E, Guernsey JM, Rosenberg SA, et al: The value of laparotomy and splenectomy in the staging of Hodgkin’s disease. Cancer 24:709-718,1969

3. Schneeberger AL, Girvan DP: Staging laparotomy for Hodgkin’s disease in children. J Pediatr Surg 23:714-717, 1988

4. Dearth JC, Gilchrist GS, Telander RL, et al: Partial splenec-

tomy for staging Hodgkin’s disease: Risk of false-negative results. N Engl J Med 299:345-346,1978

5. Sterchi JM, Buss DH, Beyer FC: The risk of improperly staging Hodgkin’s disease with partial splenectomy. Am Surg 50:20-22,1984

6. Boles ET, Haase GM, Hamoudi AB: Partial splenectomy in staging laparotomy for Hodgkin’s disease: An alternative ap- preach. J Pediatr Surg 13:581-585,1978

7. Duchesne G, Crow J, Ashley S, et al: Changing patterns of relapse in Hodgkin’s disease. Br J Cancer 60:227-230,1989