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

Syeda Iqra Usman; Kafil Akhtar; Bilal Hussain; Shahid Ali Siddiqui

doi:10.4103/aort.aort_13_22

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 2 | Page : 97-100

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

Syeda Iqra Usman¹, Kafil Akhtar¹, Bilal Hussain², Shahid Ali Siddiqui²
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 Submission	11-May-2022
Date of Decision	13-Jun-2022
Date of Acceptance	14-Jun-2022
Date of Web Publication	18-Nov-2022

Correspondence Address:
Dr. 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/aort.aort_13_22

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 2023 Mar 25];2:97-100. Available from: http://www.aort.info/text.asp?2022/2/2/97/361488

Introduction

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

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

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.

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