Classic hodgkin lymphoma involving spleen, stomach, and pancreas without peripheral lymphadenopathy: Report of a very rare case

Sharath K Krishnan; Bhavya P Mohan; Ravindran Chirukandath; M Feroze

doi:10.4103/ljms.ljms_46_21

CASE REPORT

Year : 2022 | Volume : 6 | Issue : 1 | Page : 23-26

Classic hodgkin lymphoma involving spleen, stomach, and pancreas without peripheral lymphadenopathy: Report of a very rare case

Sharath K Krishnan¹, Bhavya P Mohan², Ravindran Chirukandath³, M Feroze⁴
¹ Department of Surgical Oncology, Government Medical College, Thrissur, India
² Department of Pathology, Government Medical College, Thrissur, India
³ Department of General Surgery, Government Medical College, Thrissur, India
⁴ Department of Pathology, MES Medical College, Malappuram, Kerala, India

Date of Submission	20-Jul-2021
Date of Acceptance	10-Jun-2022
Date of Web Publication	11-Aug-2022

Correspondence Address:
Dr. Bhavya P Mohan
Department of Pathology, Government Medical College, Thrissur, Kerala
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ljms.ljms_46_21

Abstract

Classic Hodgkin lymphoma (HL) is a lymphoid neoplasm most often affecting the lymph nodes of the cervical region (75%), followed by mediastinal, axillary, and para-aortic areas. Extranodal involvement is much less common in HL. We present an extremely rare case of classic HL (CHL) with involvement of the spleen, stomach, and pancreas and perigastric, mesenteric, and para-aortic lymph nodes without peripheral lymphadenopathy, in a 54-year-old male, which clinicoradiologically mimicked a carcinoma. An extensive English literature search did not reveal any case of CHL involving the spleen, stomach, and pancreas simultaneously.

Keywords: Hodgkin, pancreas, primary, spleen, stomach

How to cite this article:
Krishnan SK, Mohan BP, Chirukandath R, Feroze M. Classic hodgkin lymphoma involving spleen, stomach, and pancreas without peripheral lymphadenopathy: Report of a very rare case. Libyan J Med Sci 2022;6:23-6

How to cite this URL:
Krishnan SK, Mohan BP, Chirukandath R, Feroze M. Classic hodgkin lymphoma involving spleen, stomach, and pancreas without peripheral lymphadenopathy: Report of a very rare case. Libyan J Med Sci [serial online] 2022 [cited 2023 Mar 28];6:23-6. Available from: https://www.ljmsonline.com/text.asp?2022/6/1/23/353692

Introduction

Hodgkin lymphoma (HL) is a malignant lymphoid neoplasm with the production of large tumor masses containing diagnostic Reed–Sternberg cells (RS cells). The REAL classification (1994), which is carried forward to the 2017 World Health Organization classification of HL, separates HL broadly into classic HL (CHL) and nodular lymphocyte-predominant HL subtypes based on the immunophenotypic and genotypic differences.^[1] CHL includes nodular sclerosis, mixed cellularity, lymphocyte depleted, and lymphocyte-rich subtypes.

HL usually presents with painless peripheral adenopathy involving cervical and supraclavicular nodes; less frequently, with involvement of axillary and inguinal nodes. Constitutional symptoms, including fever, night sweats, and weight loss, may be present. Adjacent nodal groups are usually affected as the disease progresses.

Involvement of extranodal sites, such as the spleen, liver, or bone marrow, is less common in HL than in non-HL (NHL). The RS cells of CHL usually express CD15, CD30, and are negative for CD45 by paraffin immunohistochemistry (IHC). However, since other malignancies such as anaplastic large cell lymphoma (ALCL), undifferentiated carcinoma, peripheral T-cell lymphoma, and diffuse large B-cell lymphoma (DLBCL) have similar histopathological features or Reed–Sternberg (RS) like cells, careful confirmation is necessary through the use of appropriate IHC markers.

Case Report

A 54-year-old male patient presented with dull aching abdominal pain of 3-month duration. He had associated loss of weight and loss of appetite. There was no history of vomiting, melena, fever, altered bowel habits, or night sweats. He was a chronic smoker for 35 years, which he stopped before 1 year. Neither he was alcoholic nor there any known comorbidities or previous surgeries. There was no significant family history. On physical examination, there was mild pallor. No icterus, edema, or lymphadenopathy was noted. Abdominal examination showed hepatosplenomegaly. No ascites was there.

A contrast-enhanced computed tomography (CECT) scan of the abdomen (March 2018) showed nodular enhancing lesions in the body of the stomach along greater curvature, enhancing lesions in the spleen, and segments III and IV B of the liver [Figure 1]a. Upper gastrointestinal endoscopy revealed an ulcer in cardia with nodularity involving greater curvature. Biopsy performed from this area was sent for histopathological evaluation and was reported as adenocarcinoma, diffuse type. The patient received six cycles of epirubicin, cisplatin, and 5-FU chemotherapy.

Figure 1: (a) CECT abdomen and pelvis showing nodular enhancing lesions in the body of stomach (along greater curvature), liver (segment IV B – 3.3 cm, segment III- 1.5 cm) and spleen (1.1 cm), (b) Post chemotherapy CECT showing tumor infiltration in stomach, splenic hilum and pancreas (red arrow) with absence of liver lesions, (c) Radical resection specimen (Total gastrectomy, distal pancreatectomy, Splenectomy, omentum and nodes), (d) Gross specimen showing tumor infiltration in stomach, splenic hilum (arrow) (e) Gross specimen showing tumor infiltration in pancreas (arrow)

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Upon re-evaluation after 6 months, laboratory investigations showed Hb – 8.9 g/dl, white blood cell count – 7300 cells/mm³, platelet count – 2.9lakhs/mm³, hematocrit – 28%, and erythrocyte sedimentation rate – 100 mm/h. Peripheral smear, liver enzymes, serum renal parameters and electrolytes, and coagulation profile all were normal. His chest X-ray was within normal limits. HIV screening was negative.

Postchemotherapy CECT abdomen (August 2018) showed a splenic hilar mass measuring 5 cm × 3 cm and encasing splenic vessels, irregular enhancing lesion in the body, and greater curvature of the stomach, multiple perigastric, peripancreatic, mesenteric, and para-aortic nodes, omental mass measuring 2.4 cm × 2.1 cm, abutting pylorus, and tail of the pancreas involvement. There were no focal lesions in segments III and IV B of the liver [Figure 1]b. In view of the chemotherapy response, after multidisciplinary tumor board discussion, a total gastrectomy with splenectomy and distal pancreatectomy was planned and the patient underwent the surgery in October 2018 [Figure 1]c. Intraoperative findings were correlating with CECT and the major mass lesion was in the region of proximal greater curvature extending to the splenic hilum and was infiltrating the distal part of the pancreas close to the splenic hilum.

In our histopathology laboratory, grossly, in the gastrectomy specimen, we noted a nodular growth on the greater curvature measuring 4 cm × 2.5 cm × 1.5 cm and ulcer in the greater curvature measuring 4.2 cm × 3.8 cm × 0.3 cm. The spleen and pancreas were adherents to the stomach wall [Figure 1]d. In the spleen, a gray-white lesion measuring 5 cm × 5 cm × 4 cm was noted. The pancreas showed a gray-white lesion measuring 5.5 cm × 2.8 cm × 2 cm [Figure 1]e. A separately received piece of the pancreas also showed a similar appearance which measured 4 cm × 2.5 cm × 0.6 cm. Forty lymph nodes (perigastric, peripancreatic, mesenteric, and para-aortic) were identified; some were matted, with largest one measuring 1.4 cm in diameter.

Histologically, sections from the stomach, spleen, pancreas, and lymph nodes (23 out of 40) showed a similar appearance of an infiltrating neoplasm composed of cellular infiltrate of lymphocytes, plasma cells, histiocytes and eosinophils, and many RS cells. Occasional Hodgkin cells, pleomorphic forms, and mummified cells were noted. Gastric mucosal ulceration was seen with normal mucosa in adjacent areas. IHC wise, RS cells and variants showed positivity for CD30 and CD15 antigens and negativity for CD45, ALK-1, CD3, CD20, and CK [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d,[Figure 2]e,[Figure 2]f. Small lymphocytes in the background were positive for CD45. On the basis of clinicopathological features and IHC, a definitive diagnosis of CHL, mixed cellularity type, involving the spleen, stomach, pancreas and perigastric, peripancreatic, mesenteric, and para-aortic lymph nodes, was established. (Previous gastric biopsy slide was reviewed and IHC was performed on its paraffin block, which was keeping in with HL). Without clinicoradiological evidence of any peripheral lymphadenopathy and with positive para-aortic nodes, a CHL with simultaneous involvement of the spleen, stomach, and pancreas with perigastric, peripancreatic, and mesenteric nodes was considered.^[2]

Figure 2: (a) Microscopy of stomach, spleen, pancreas, perigastric (D1 and D2) and para-aortic lymph nodes showing neoplastic mononuclear Hodgkin and bi/multinucleated Reed Sternberg cells in a background of inflammatory cells (Inset: Reed Sternberg cells) (H&E x 400), (b)CK negative neoplastic cells (IHC x 400), (c) LCA negative neoplastic cells (IHC x 400) (d) CD30 positive neoplastic cells (IHC x 400), (e) CD15 positive neoplastic cells (IHC x 400), (f)ALK negative neoplastic cells (IHC x 400)

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The patient was then started on appropriate chemotherapy. He responded clinically with interval positron emission tomography scan showing almost complete resolution of the mass lesions. He had completed six cycles of chemotherapy and was available for follow-up till 1 year, after which he defaulted.

Discussion

The majority of primary lymphoid malignancies of the gastrointestinal tract, especially the stomach, are constituted by NHL.^[3] Primary splenic lymphoma is a rare neoplasm of the spleen, comprising <2% of all lymphomas, and 1% of all the NHL. Less than 2% of all extranodal malignant lymphomas and 0.5% of all pancreatic masses are primary pancreatic lymphomas.^[4] Primary HL of the stomach and spleen is very rare.^[5],[6] The pancreas involvement in CHL is also extremely rare.^[2]

CHL most often involves lymph nodes of the cervical region (75% of cases), followed by the mediastinal, axillary, and para-aortic regions. Nonaxial lymph node groups, such as mesenteric and epitrochlear lymph nodes, are rarely involved.^[2] Extranodal involvement is much less common in HL (<1%) than in NHL. The most common site of involvement is the gastrointestinal tract, followed by (in order of descending frequency) the pulmonary system, thyroid, skin, genitourinary system, and central nervous system.^[4] In mixed cellularity CHL (MCHL), the spleen is involved in 30% of cases, bone marrow in 10%, liver in 3%, and other organs in 1%–3%.^[2] Splenic involvement is associated with an increased risk of extranodal dissemination.

As cases of primary gastrointestinal HL are so rare, the diagnosis should only be made following strict histologic and other criteria proposed by Dawson et al., that is (a) no superficial lymphadenopathy should be present at the time of diagnosis, (b) chest radiological studies should indicate no involvement of mediastinal lymph nodes, (c) the complete blood count and white cell differential should be within normal limits, (d) gastrointestinal lesion should predominate with or without positive adjacent lymph nodes, and (e) the liver and spleen should be free of disease at the time of diagnosis.^[7] In our case, as the spleen was involved and para-aortic nodes demonstrated the tumor, we considered the possibility of CHL with secondary, simultaneous involvement of the spleen, stomach, and pancreas with perigastric, peripancreatic, and mesenteric nodes.

Most cases of MCHL show a diffuse obliteration of nodal architecture without capsular thickening or broad bands of parenchymal fibrosis. Classic RS cells are easily found, often in a background of small lymphocytes, eosinophils, histiocytes, plasma cells, or neutrophils. Classic RS is derived from germinal center B cells or activated postgerminal center B cells with absent or weak expression of most B-cell-associated immunophenotypic markers.

Morphologically, the differentials of our case were ALCL, anaplastic variant of DLBCL, and undifferentiated carcinoma. By IHC staining, our patient demonstrated large atypical cells with RS morphology showing membranous CD30 and CD15 positivity and CD45, CK, ALK-1, CD3, and CD20 negativity. We thus excluded the diagnosis of undifferentiated carcinoma, ALCL, and the anaplastic variant of DLBCL, confirming the diagnosis of CHL.

In conclusion, we have presented an extremely rare case of CHL with simultaneous involvement of the spleen, stomach, and pancreas which mimicked a carcinoma. Judicious use of IHC in gastric malignancies with a diffuse infiltrate of malignant cells is suggested to confirm/exclude lymphoma which is crucial in subsequent patient management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. A revised European-American classification of lymphoid neoplasms: A proposal from the international lymphoma study group. Blood 1994;84:1361-92.
2.	Swerdlow SH, Campo E, Haris NL, Jaffe ES, Pileri SA, Stein H, et al. World Health Organization Classification of Tumours of the Hematopoietic and lymphoid Tissues. 4^th ed., Lyon, France: International Agency for Research on Cancer; 2017. p. 425-40.
3.	Haji AG, Sharma S, Majeed KA, Vijaykumar DK, Pavithran K, Dinesh M. Primary pancreatic lymphoma: Report of three cases with review of literature. Indian J Med Paediatr Oncol 2009;30:20-3. [PUBMED] [Full text]
4.	Gandhi JS, Mehta A, Sharma A, Kamboj M. Primary Hodgkin lymphoma of the ileum. J Cancer Res Ther 2010;6:342-3.
5.	Isaacson NH, Spatt SD, Grayzel DM. Primary splenic Hodgkin's disease without lymph node involvement. Ann Intern Med 1947;27:294-301.
6.	Hossain FS, Koak Y, Khan FH. Primary gastric Hodgkin's lymphoma. World J Surg Oncol 2007;5:119.
7.	Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;49:80-9.