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| CASE REPORT |
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| Year : 2022 | Volume
: 6
| Issue : 2 | Page : 56-58 |
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A rare case of primary Hodgkin's lymphoma of the pancreas
Andleeb Abrari1, Durre Shehwar2, Bilal Hussain3, Kafil Akhtar2
1 Department of Pathology, The Rotherham NHS Foundation Trust, Rotherham, United Kingdom
2 Department of Pathology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
3 Department of Radiotherapy and Clinical Oncology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
| Date of Submission | 15-Jun-2022 |
| Date of Decision | 11-Aug-2022 |
| Date of Acceptance | 12-Aug-2022 |
| Date of Web Publication | 29-Aug-2022 |
Correspondence Address:
Kafil Akhtar
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/oji.oji_19_22
Primary pancreatic lymphoma is an extremely rare entity presenting 2.0% of extranodal malignant lymphomas and 0.5% of all pancreatic masses. We present the case of primary pancreatic Hodgkin's lymphoma in a 62-year-old male, who presented with right hypochondrial pain, nausea, vomiting, yellowish discoloration of skin and sclera, accompanied by generalized itching for 3 weeks without any significant loss of appetite and weight loss. Endoscopic ultrasound (EUS) showed a reddish bulge in the head of the pancreas due to the growth. EUS-guided biopsy revealed extensive infiltration by dyscohesive lymphoid cells with classic morphology of mononuclear Reed–Sternberg cells, in a background of lymphocytes and eosinophils. This rare report will be of significant diagnostic help to clinicians and pathologists in accurately diagnosing such cases as there is a high likelihood of misinterpreting these as pancreatic adenocarcinoma, which may impact patient management and survival.
Keywords: Endoscopic biopsy, Hodgkin's lymphoma, immunohistochemistry, pancreatic, primary
How to cite this article:
Abrari A, Shehwar D, Hussain B, Akhtar K. A rare case of primary Hodgkin's lymphoma of the pancreas. Oncol J India 2022;6:56-8 |
| Introduction | |  |
Nodal disease in Hodgkin's lymphoma is the norm, with most patients presenting at early stages and well amenable to currently defined management strategies.[1] Extranodal tissue involvement is rarely seen with Hodgkin's lymphoma, whereas non-Hodgkin's lymphoma often involves extranodal sites. The conceptual definition of primary extranodal lymphomas is controversial, although as accepted presently for practice purposes, it is allowed to consider a lymphoma as extranodal with none or only “minor” nodal, splenic, or bone marrow involvement in conjunction with a clinically dominant extranodal component.
Primary pancreatic lymphoma (PPL) is an extremely rare entity presenting 2.0% of extranodal malignant lymphomas and 0.5% of all pancreatic masses.[1] There are very few cases of primary extranodal pancreatic Hodgkin's lymphoma reported mainly as stray case reports.[2],[3] The diagnostic criteria for PPL includes a mass predominantly located within the pancreas, with grossly involved lymph nodes confined to the peripancreatic region, no palpable superficial and mediastinal lymphadenopathy, no hepatic or splenic involvement, and normal leukocyte count. They are clinically liable to be mistaken for pancreatic adenocarcinoma and radiology is not helpful in differentiating these.[4] Histopathologic examination is required for a definitive diagnosis. Owing to the differences in prognosis and management, it is essential to differentiate these.
| Case Report | | |
A 62-year-old male presented with right hypochondrial pain, nausea, vomiting, and yellowish discoloration of skin and sclera, accompanied by generalized itching for 3 weeks without any significant loss of appetite and weight loss. There was no significant family and drug history. General examination revealed icterus and abdominal tenderness. Cervical, axillary, or another peripheral lymphadenopathy with organomegaly was not seen.
The liver function test showed derangement with a total bilirubin level of 11.7 mg/dL, a lactate dehydrogenase level of 323 U/L, and a beta-2 microglobulin level of 5.7 mg/L. CA 19-9 levels were not raised. Contrast-enhanced computed tomography (CECT) scan of the abdomen revealed a large mass of size 52 mm × 36 mm mass involving the head of the pancreas and uncinate process, closely abutting the D2 segment of the duodenum, with dilated intrahepatic and common bile ducts [Figure 1]. No associated intra-abdominal or retroperitoneal lymphadenopathy was seen. Endoscopic retrograde cholangiopancreatography revealed irregular narrowing of the pancreatic duct with dilated proximal segment. Endoscopic ultrasound (EUS) showed a reddish bulge in the head of the pancreas due to the growth [Figure 2]. EUS-guided biopsy from the growth on microscopic examination with hematoxylin and eosin stain revealed extensive infiltration by dyscohesive lymphoid cells with classic morphology of mononuclear Reed–Sternberg (RS) cells, in a background of lymphocytes and eosinophils [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d. On immunohistochemistry, RS cells showed CD 30 [Figure 4]a and CD 15 positivity. There was the presence of characteristics rossetting of typical a macronucleolated Hodgkin RS cell by CD3 marked lymphocytes [Figure 4]b. CECT scan of the neck, thorax, and pelvis was unremarkable. The liver and spleen were normal in size and texture. Bone marrow examination did not show any foci of tumor involvement. With the above histopathological and immunohistochemical findings, a diagnosis of primary pancreatic Hodgkin's lymphoma, Stage IB (T2N0M0) was confirmed. To relieve the obstructive symptomatic features of jaundice, our patient underwent pancreatectomy with biliary-intestinal anastomotic surgery followed by adjuvant chemotherapy with six cycles of COPP regimen (cyclophosphamide 50 mg, oncovin 40 mg, procarbazine 40 mg, and prednisolone 60 mg). He is well with no foci of recurrence after 12 months of the follow-up period. | Figure 1: CECT scan of the abdomen showing a mass of size 52 mm × 36 mm involving the head of the pancreas and uncinate process. CECT: Contrast-enhanced computed tomography
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 | Figure 2: EUS showing a reddish bulge in the head of the pancreas due to the growth (*). EUS: Endoscopic ultrasound
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 | Figure 3: (a-d) Microscopic examination of hematoxylin and eosin staining reveals pancreatic parenchyma abutted and infiltrated by polymorphous cellularity (a: ×10), and cellularity typical for classical Hodgkin's lymphoma with the presence of RS cells admits characteristics lympho-leukocytic background (b-d: ×40)
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 | Figure 4: (a and b) H-RS cells expressing IHC CD30 (a: ×40), and characteristic rosetting of typical a macronucleolated H-RS cell by CD3 marked T lymphocytes (b: ×40)
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| Discussion | | |
Here, we are presenting the first case of its kind of primary pancreatic extranodal Hodgkin's lymphoma, which to the best of our knowledge has not yet been described in any English literature. Ma et al. reported that in 26 out of 251 consecutive Hodgkin's lymphoma cases, the involvement was “primary extranodal” – with the respiratory system and gastrointestinal tract being the sites of predilection prominently.[5] They did not encounter a single case of primary extranodal pancreatic Hodgkin's lymphoma in their study.[5]
Significantly, almost all cases of PPL reported to date have been non-Hodgkin's B-cell lymphomas. There are very few cases of primary extranodal pancreatic Hodgkin's lymphoma reported mainly as stray case reports.[2],[3] Das et al. maintained that a primary extranodal lymphoma of the pancreas is rare and usually a non-Hodgkin's lymphoma.[6] They also aver that the majority of pancreatic lymphomas are secondary, resulting from contiguous spread from nodal disease in the vicinity anatomically abetted by the lack of a true capsule of the pancreas. Significantly, they did not report even a single case of primary extranodal pancreatic Hodgkin's lymphoma in their series of 281 cases.[6]
Clinical presentations of patients with primary extranodal Hodgkin's lymphoma depend on the organ involved. The absence of peripheral lymphadenopathy was a consistent finding in several studies.[5],[7] This warrants thorough and repeated investigations to reach a conclusive diagnosis. Hence, CT and EUS-guided biopsies are of significant help. It is essential to diagnose these early, as extranodal Hodgkin's lymphomas have a better outcome.[6],[8] Their management should be similar to nodal Hodgkin's lymphoma and they should not be confused with the similar pathologies of that site.
| Conclusions | | |
This rare report will be of significant diagnostic help to clinicians and pathologists in accurately diagnosing such cases, as there is a high likelihood of misinterpreting these as pancreatic adenocarcinoma, which may impact patient management and survival.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Acknowledgments
The authors would like to thank the histopathology laboratory staff and Mr. Raju Mehdi (SLA) for the immunohistochemistry slides.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Iyengar P, Mazloom A, Shihadeh F, Berjawi G, Dabaja B. Hodgkin lymphoma involving extranodal and nodal head and neck sites: Characteristics and outcomes. Cancer 2010;116:3825-9.  |
| 2. | Mohamadnejad M, Khosravi P, Khani M, Nikmanesh A, Eloubeidi MA. Primary pancreatic Hodgkin's lymphoma diagnosed on EUS-guided FNA. Gastrointest Endosc 2016;83:844-5. |
| 3. | Facchinelli D, Boninsegna E, Visco C, Tecchio C. Primary pancreatic lymphoma: Recommendations for diagnosis and management. J Blood Med 2021;12:257-67. |
| 4. | Baysal B, Kayar Y, Ince AT, Arici S, Türkmen I, Şentürk H. Primary pancreatic lymphoma: A rare cause of pancreatic mass. Oncol Lett 2015;10:1701-3. |
| 5. | Ma J, Wang Y, Zhao H, Liu S, Li Q, Lin L, et al. Clinical characteristics of 26 patients with primary extranodal Hodgkin lymphoma. Int J Clin Exp Pathol 2014;7:5045-50. |
| 6. | Das J, Ray S, Sen S, Chandy M. Extranodal involvement in lymphoma-A pictorial essay and retrospective analysis of 281 PET/CT studies. Asia Ocean J Nucl Med Biol 2014;2:42-56. |
| 7. | Khalid S, Zaheer S, Khalid M, Zaheer S, Alam MS. Primary liver lymphoma. JBR-BTR 2013;96:84-6. |
| 8. | Lin H, Li SD, Hu XG, Li ZS. Primary pancreatic lymphoma: Report of six cases. World J Gastroenterol 2006;12:5064-7. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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