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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 97-100

Synchronous Hodgkin's lymphoma and periampullary carcinoma: An unusual association


1 Department of Pathology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Radiotherapy, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Date of Submission11-May-2022
Date of Decision13-Jun-2022
Date of Acceptance14-Jun-2022
Date of Web Publication18-Nov-2022

Correspondence Address:
Dr. Kafil Akhtar
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aort.aort_13_22

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  Abstract 

Carcinomas arising within 2.0 cm of major duodenal papilla are called periampullary carcinomas. These include carcinomas of the ampulla, distal common bile duct, pancreas, and duodenum. Hodgkin's lymphoma is a malignant neoplasm of lymphoreticular cell origin characterized by the presence of Hodgkin's and Reed–Sternberg cells. We report the case of a 58-year-old male with a 9-month history of abdominal pain, nausea, and vomiting with jaundice and itching all over the body for the past 4 months along with generalized lymphadenopathy and fever for the past 7 months. On ultrasonography, a periampullary mass of size 6.6 cm × 3.5 cm was noted. On computed tomography, an ill-defined soft-tissue mass measuring 7 cm × 3.5 cm × 1.8 cm was seen in the periampullary region with marked dilatation of common bile duct and generalized lymphadenopathy. Fine-needle aspiration and biopsy from both the mass lesions were performed. The patient underwent the Whipple procedure. He was diagnosed with synchronous periampullary carcinoma of the pancreas with Hodgkin's lymphoma based on radiological and histopathological examination of resected mass and excisional biopsy of a lymph node.

Keywords: Hodgkin's lymphoma, pancreatic carcinoma, periampullary, Reed–Sternberg cells


How to cite this article:
Usman SI, Akhtar K, Hussain B, Siddiqui SA. Synchronous Hodgkin's lymphoma and periampullary carcinoma: An unusual association. Ann Oncol Res Ther 2022;2:97-100

How to cite this URL:
Usman SI, Akhtar K, Hussain B, Siddiqui SA. Synchronous Hodgkin's lymphoma and periampullary carcinoma: An unusual association. Ann Oncol Res Ther [serial online] 2022 [cited 2022 Nov 29];2:97-100. Available from: http://www.aort.com/text.asp?2022/2/2/97/361488


  Introduction Top


Periampullary carcinomas have an incidence of 0.5%–2.0% of all gastrointestinal malignancies.[1],[2] These are tumors within 2.0 cm of the major duodenal papilla and include tumors arising from the ampulla of Vater (20%–30.0%), intrapancreatic distal bile duct (10.0%), head and uncinate process of the pancreas (50%–60.0%), and duodenum (<10.0%).[3] Clinical symptoms vary due to different locations but jaundice is one of the common symptoms.[4] The five-year survival rate of periampullary carcinoma is reported to be 15%–65.0%.[5]

Hodgkin's lymphoma is a B-cell-derived neoplasm and constitutes 15%–25.0% of all lymphomas. It is characterized by mononuclear Hodgkin's cells and Reed–Sternberg cells.[6] It is the most common neoplasm in young adults and has bimodal presentation one in the third decade and the other after 55 years of life.[7] Epstein–Barr virus latent infection has been identified in 50.0% of Hodgkin's lymphomas.[8]


  Case Report Top


A 58-year-old male presented to the surgical outpatient department with complaints of continuous and dull aching abdominal pain and nausea and vomiting for the past 9 months. He also complained of multiple small swellings over his body which first started in the cervical region at 7 months. There was a history of on and off fever for 7 months and yellowish discoloration of eyes and itching all over the body for the past 4 months. He also had a loss of weight and appetite. There was no history of any comorbidity. None of the family members had similar complaints. The patient was a chronic smoker for the past 25 years. There was no history of any drug intake or blood transfusion. There was no previous treatment history.

On examination, the patient was pale and icteric. There was tender hepatomegaly 4.0 cm below the right subcostal margin. The spleen was not enlarged. There were multiple firms, enlarged tender, and mobile lymph nodes in the cervical, axillary, and inguinal regions. Multiple excoriation marks were present all over the body.

Investigations showed microcytic hypochromic anemia (hemoglobin – 6.0 gm%) with leukocyte count of 13500 cells/mm3 and neutrophils were 87% in the differential. The liver function test was deranged, with total and direct bilirubin of 5.8 mg/dl and 5.0 mg/dl, respectively. Alkaline phosphatase was 655 U/L. Ultrasonography of the abdomen revealed a liver size of 17 cm and dilatation of the common bile duct. A heterogeneous hyperechoic mass was noted in the periampullary region of 6 cm × 3.5 cm in size. On contrast-enhanced computed tomography of the abdomen, a heterogeneous hyperechoic mass was noted near the head of the pancreas with a size of 7 cm × 3.5 cm × 1.8 cm. Magnetic resonance cholangiopancreatography showed a well-defined lesion in the region of the head and uncinate process of the pancreas with obstruction of the common bile duct features suggestive of a malignant lesion.

The patient was evaluated for lymphadenopathy and fine-needle aspiration was performed which showed a good cellularity smear with scattered Reed–Sternberg cells having multilobed nuclei and prominent nucleoli in the background of mixed inflammatory infiltrate [Figure 1]. Excisional biopsy of lymph node showed distorted lymph node architecture with some scattered classical Reed–Sternberg cells and some mononuclear Hodgkin's cells in the background of small lymphocytes and eosinophils [Figure 2]. Immunohistochemistry showed Hodgkin's and Reed–Sternberg cells were strongly positive for CD30 [Figure 3] and CD15 and weakly positive for PAX5. CD45, CD20, and CD3 were negative.
Figure 1: Fine-needle aspiration from the enlarged lymph node showed good cellularity smear with scattered Reed–Sternberg cells having multilobed nuclei and prominent nucleoli in the background of mixed inflammatory infiltrate. H and E, ×40

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Figure 2: Excisional biopsy of lymph node showed distorted lymph node architecture with some scattered classical Reed–Sternberg cells and some mononuclear Hodgkin's cells in the background of small lymphocytes and eosinophils. H and E, ×40

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Figure 3: Immunohistochemistry of lymphoid tissue biopsy showed Hodgkin's and Reed–Sternberg cells were strongly positive for CD30. IHC CD30, ×40

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Fine-needle aspiration cytology of the periampullary mass showed round-to-oval atypical cells in acinar configuration [Figure 4]. Whipple procedure was performed and the specimen showed a solid, gray mass of size 7 cm × 4 cm × 2 cm in the head of the pancreas with foci of necrosis. Microscopic examination revealed atypical cells with hyperchromatic nucleus and scant cytoplasm with acinar formation [Figure 5]. On immunohistochemistry, the tumor cells were strongly positive for CK7 [Figure 6]. Based on history, clinical examination, and investigations, the patient was diagnosed with a case of periampullary carcinoma with Hodgkin's lymphoma. Postoperatively, the patient was given six cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine at an interval of 21 days. The patient succumbed after 6 months of ending treatment.
Figure 4: Fine-needle aspiration cytology of the periampullary mass showed round-to-oval atypical cells in acinar configuration. H and E, ×40

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Figure 5: Microscopic examination of the periampullary mass revealed atypical cells with a hyperchromatic nucleus and scant cytoplasm with acinar formation. H and E, ×40

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Figure 6: On immunohistochemistry, the tumor cells were strongly positive for CK7. IHC CK7, ×40

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  Discussion Top


Synchronous presentation is defined as two primary cancers occurring within 6 months. Due to infrequent occurrences, their accurate diagnosis is difficult. The accurate treatment plan for these tumors is unclear.

Periampullary carcinomas are divided into two main histological types, i.e., pancreatobiliary and intestinal.[9] Pancreatobiliary histological type is independently a poor prognostic factor. In pancreatobiliary type adenocarcinomas, survival is dependent on tumor size, regional lymph node involvement, and distant metastasis but not on the anatomic structure of origin.[10] The five-year survival rate of patients having pancreatic adenocarcinoma is very low (20.0%) even after pancreatic resection. The five-year survival rate for patients who have periampullary malignancies other than pancreatic adenocarcinoma is 61.0% after pancreatoduodenectomy.[11]

It is difficult to diagnose the exact site of periampullary carcinoma preoperatively. A multicentre study of periampullary tumors showed that 13.0% of suspected cases of pancreatic cancer had a nonpancreatic periampullary tumor and 21.0% of the suspected cases of nonpancreatic periampullary tumors came out to be pancreatic in origin postoperatively.[12] The presence of precursor high-grade intraepithelial lesions may be a useful hint for the exact site of origin. High-grade pancreatic intraepithelial neoplasia is more common in pancreatic cancer, whereas high-grade biliary intraepithelial neoplasia is found commonly in distal bile duct cancer than in ampullary, pancreatic, and duodenal cancers.[13]

Hodgkin's lymphoma usually involves cervical, mediastinal, supraclavicular, and axillary lymph nodes.[14] Primary extranodal disease is rare and extranodal involvement occurs by hematogenous dissemination. The most commonly involved extranodal sites include the lung, liver, and bone.[14] Poor prognostic factors of Hodgkin's lymphoma include large mediastinal mass, involvement of three or more lymph nodes, high erythrocyte sedimentation rate, extranodal lesions, B symptoms, and advanced age.[13],[14] Tumor microenvironment has been associated with prognosis. The number of tumor-associated macrophages is associated with refractoriness to therapy, early relapse, and shortened survival.[12],[14] The patient's age, symptoms, and tumor stage are important factors considered while deciding the treatment strategy.[15]

Our patient was diagnosed with a case of periampullary adenocarcinoma (stage III), with pancreatobiliary type histological differentiation. Based on excisional biopsy and clinical findings, our patient also suffered from synchronous classical Hodgkin's lymphoma-lymphocyte predominant and stage IIIB of Ann Arbor classification. Whipple procedure was performed and six cycles of Cyclophosphamide, Oncovin, Procarbazine, Prednisolone (COPP) chemotherapy were administered at an interval of 21 days.

He was admitted at 6 months of follow-up with complaints of persistent fever, vomiting, and difficulty in breathing but unfortunately, succumbed to death. This is by far to the best of our knowledge, the first case of synchronous presentation of Hodgkin's lymphoma with periampullary carcinoma.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Uomo G. Periampullary carcinoma: Some important news in histopathology. JOP 2014;15:213-5.  Back to cited text no. 1
    
2.
Kennedy-Nasser AA, Hanley P, Bollard CM. Hodgkin disease and the role of the immune system. Pediatr Hematol Oncol 2011;28:176-86.  Back to cited text no. 2
    
3.
Sarmiento JM, Nagomey DM, Sarr MG, Farnell MB. Periampullary cancers: Are there differences? Surg Clin North Am 2001;81:543-55.  Back to cited text no. 3
    
4.
Liang YM, Wang T, Sun QL, Cheng YF. A comparison of the clinical features and prognosis of pancreatic head cancer with that of other periampullary cancers. Hepatogastroenterology 2013;60:864-9.  Back to cited text no. 4
    
5.
Sughayer MA, Haddad HA, Al-Yousef RM, El-Khateeb M, Abu-Rass H. Epstein-barr virus and Hodgkin lymphoma in Jordan. Hematol Oncol Stem Cell Ther 2014;7:85-9.  Back to cited text no. 5
    
6.
Chen JW, Bhandari M, Astill DS, Wilson TG, Kow L, Brooke-Smith M, et al. Predicting patient survival after pancreaticoduodenectomy for malignancy: Histopathological criteria based on perineural infiltration and lymphovascular invasion. HPB (Oxford) 2010;12:101-8.  Back to cited text no. 6
    
7.
Baghmar S, Agrawal N, Kumar G, Bihari C, Patidar Y, Kumar S, et al. Prognostic factors and the role of adjuvant treatment in periampullary carcinoma: A single-centre experience of 95 patients. J Gastrointest Cancer 2019;50:361-9.  Back to cited text no. 7
    
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Armengol M, Santos JC, Fernández-Serrano M, Profitós-Pelejà N, Ribeiro ML, Roué G. Immune-checkpoint inhibitors in B-cell lymphoma. Cancers (Basel) 2021;13:E214.  Back to cited text no. 8
    
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Bröckelmann PJ, Eichenauer DA, Jakob T, Follmann M, Engert A, Skoetz N. Hodgkin lymphoma in adults. Dtsch Arztebl Int 2018;115:535-40.  Back to cited text no. 9
    
10.
van Roessel S, Soer EC, Daamen LA, van Dalen D, Fariña Sarasqueta A, Stommel MW, et al. Preoperative misdiagnosis of pancreatic and periampullary cancer in patients undergoing pancreatoduodenectomy: A multicentre retrospective cohort study. Eur J Surg Oncol 2021;47:2525-32.  Back to cited text no. 10
    
11.
Jeong BK, Sung YN, Kim SJ, An S, Park H, Hwang HS, et al. High-grade precursor lesions can be used as surrogate markers to identify the epicenter of periampullary carcinomas. Hum Pathol 2019;84:92-104.  Back to cited text no. 11
    
12.
Laurent C, Do C, Gourraud PA, de Paiva GR, Valmary S, Brousset P. Prevalence of common non-Hodgkin lymphomas and subtypes of Hodgkin lymphoma by nodal site of involvement: A systematic retrospective review of 938 cases. Medicine (Baltimore) 2015;94:e987.  Back to cited text no. 12
    
13.
Aldin A, Umlauff L, Estcourt LJ, Collins G, Moons KG, Engert A, et al. Interim PET-results for prognosis in adults with Hodgkin lymphoma: A systematic review and meta-analysis of prognostic factor studies. Cochrane Database Syst Rev 2019;9:CD012643.  Back to cited text no. 13
    
14.
Meng J, Pan H, Li X, Liu T, Liu Z, Li Q, et al. Diagnosis and treatment of synchronous lymphoma and digestive system carcinoma: Report of four cases and literature review. Front Oncol 2019;9:1367.  Back to cited text no. 14
    
15.
Deau B, Bachy E, Ribrag V, Delarue R, Rubio MT, Bosq J, et al. Macrophage, mast cell and T lymphocyte infiltrations are independent predictive biomarkers of primary refractoriness or early relapse in classical Hodgkin lymphoma. Leuk Lymphoma 2013;54:41-5.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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