|Year : 2020 | Volume
| Issue : 1 | Page : 24-26
An unusual presentation of non-hodgkin's lymphoma
Vishwaraj Baswaraj Tadakal, Sangram Biradar, Muddasir Indikar
Department of General Medicine, M.R. Medical College, Kalaburgi, Karnataka, India
|Date of Submission||08-Jul-2019|
|Date of Acceptance||09-Sep-2019|
|Date of Web Publication||14-Jan-2020|
Dr. Sangram Biradar
Department of General Medicine, M.R. Medical College, Kalaburgi, Karnataka
Source of Support: None, Conflict of Interest: None
Non-Hodgkin's lymphoma (NHL) causes many deaths worldwide. It represents a heterogeneous group of neoplasms originating from the lymphocytes. The etiology of this disease is still unclear. Some infectious agents have been associated more or less strictly with the development of an NHL as, i.e., the Epstein–Barr virus, the human herpesvirus-8, and the human T-cell lymphotropic virus Type I. Nearly 70% of lymphomas present a generally multilocalized, single, or multiple lymphoadenomegaly without pain, with involvement in almost 30%–40% of laterocervical lymphnodes. In 30% of patients, an extranodal localization is reported in Waldeyer's ring, in the stomach, and generally, in the gastrointestinal tract. The retroperitoneal localization is extremely rare and its diagnosis is often difficult. It often requires a time-consuming and costly diagnostic workup. Here, we present a rare case of NHL presented with retroperitoneal mass encasing the aorta.
Keywords: Aorta, Non-Hodgkin's lymphoma, retroperitoneal mass
|How to cite this article:|
Tadakal VB, Biradar S, Indikar M. An unusual presentation of non-hodgkin's lymphoma. APIK J Int Med 2020;8:24-6
|How to cite this URL:|
Tadakal VB, Biradar S, Indikar M. An unusual presentation of non-hodgkin's lymphoma. APIK J Int Med [serial online] 2020 [cited 2020 Jan 23];8:24-6. Available from: http://www.ajim.in/text.asp?2020/8/1/24/275977
| Introduction|| |
Malignant lymphoma is the generic term given to tumors of the lymphoid system, and these are divided into two major categories: Hodgkin's lymphoma (HL) and non-HL (NHL). NHL usually involves the lymph nodes but can involve extranodal sites and can occur in the stomach, skin, lung, salivary glands, and rarely, in the mouth.
NHL is a heterogeneous group of malignancies characterized by an abnormal clonal proliferation of T-cells, B-cells, or both. The majority of the adult NHLs are of B-cell origin. NHLs range from indolent malignancies (low-grade histologies) to rapidly growing and highly aggressive tumors (high-grade histologies). The overall median age at presentation is 42 years (58 years for low-grade), and the incidence increases with advancing age. The majority of NHLs are of B-cell origin. In general, low-grade or follicular NHL is assumed to have an indolent course when compared with intermediate- and high-grade NHL.
| Case Report|| |
Here, we present a 90-year-old male who had complaints of abdominal pain, difficulty in swallowing, and loss of appetite.
Initially, the patient complained of abdominal pain, dull aching type diffuse throughout the abdomen and more in the left upper quadrant region; there was no aggravating factor, and the pain subsided on taking over the counter medications; the patient also complained of difficulty in swallowing both solids and liquids and also loss of appetite for 3–4 months.
Clinical examination vitals included blood pressure: 110/70 mmHg and pulse rate: 84 bpm; general physical examination revealed gross malnutrition, poor oral hygiene, and multiple inguinal lymph nodes, with the largest noted on the right side measuring 2 cm × 1 cm; right-sided scrotal swelling was also seen. On systemic examination, a mass around the epigastric area was felt measuring roughly 8 cm × 10 cm, and the associated areas were dull; there were no evidence of ascites or obstruction, and other systemic examinations were completely normal.
Investigations revealed normal hemogram with Hb of 11 (13–17), total count of 5700 (4000–10,000), and normal peripheral smear; the patient was tested negative for HIV, HBV, and HCV, and liver and renal functions were within the normal limits. Prostate-specific antigen was 4.5 ng/ml (1.64–4.0), and serum lactic dehydrogenase was 547 IU/L (100–190).
Ultrasonography (USG) of the abdomen was suggested and was reported to have:
- Large retroperitoneal mass having mixed echogenicity size of 15 cm × 12 cm encasing the aorta and inferior vena cava and displacing the pancreas and stomach anteriorly bowel inferiorly [Figure 1]
- Multiple mesenteric and inguinal lymph nodes with altered echotexture [Figure 2] and [Figure 3].
A follow-up contrast enhancement computed tomography (CT) revealed large mass in the preaortic and para-aortic regions encasing the aorta, possibly malignant, minimal ascites and omental thickening, prostatomegaly and simple cortical cyst in right kidney.
A USG-guided biopsy was done; three linear fragments were taken, and histologically it was reported.
IHC and immunophenotyping was advised, but the patient refused due to personal problems.
The patient was started on cyclophosphamide 50 mg OD and allopurinol 100 mg Thrice a day as per the advice by an oncologist and was advised to follow-up after 10 days.
| Discussion|| |
Lymphoma is a general term for a complex group of malignancies of the lymphoreticular system. These malignancies initially arise within the lymphatic tissues and may progress to an extranodular mass (NHL) or to a nontender mass or masses in a lymph node region (HL) that later may spread to other lymph node groups and involve the bone marrow. Hodgkin's disease and NHLs are considered major types in comparison with Burkitt's lymphoma and mycosis fungoides (T-cell lymphoma involving the skin), which are rare forms of the disease. The HL and NHL are hematologic tumors, which are usually diagnosed by a biopsy of an enlarged lymph node or mass. A local mass or pain is the most common initial complaint of the majority of patients with extranodal lymphoma. NHL has long been recognized as a heterogeneous group of disorders based on clinical presentation, morphological appearance, and response to therapy. In recent years, the use of immunological and molecular biological techniques has led to important advances in our knowledge of lymphocyte differentiation and has provided the basis for a better understanding of the cellular origin and pathogenesis of NHL. Currently, various types of NHL are thought to represent neoplastic cells arrested at various stages in the normal differentiation scheme although the key events in malignant transformation may actually occur in cells at an earlier stage of differentiation.
The NHLs are a diverse group of lymphoid neoplasms that collectively rank fifth in cancer incidence and mortality. The prevalence of NHL has been increasing during the past two decades. Recognized since the 1950s as a distinct group of diseases, NHLs range from indolent malignancies (low-grade histologies) to rapidly growing and highly aggressive tumors (high-grade histologies). The majority of NHLs are of B-cell origin, with more than 90% of patients expressing CD20 antigen. In general, low-grade or follicular NHL is assumed to have an indolent course when compared with intermediate- and high-grade NHL.
The main symptom of both HL and NHL is the swelling of lymph nodes in the neck, under the arms, or in the groin. Other symptoms include fever, night sweats, fatigue, abdominal pain, and unexplained weight loss. Lymphomas usually are painless; lymph nodes may get larger slowly over a long time before the patient notices. Fever commonly associated with lymphoma may appear and disappear for several weeks.
Diffuse large B-cell lymphoma is the most common histological NHL subtype in adult patients. Although lymphomas are considered to be one of the most common malignancies of the retroperitoneum, this location has been very rarely reported as the primary site. Due to the uncommon anatomical location and the lack of symptoms in many of them for a long time, the diagnosis is usually late and the management of this patient is difficult. Although CT scan is the diagnostic modality of choice, magnetic resonance imaging offers superior soft-tissue contrast in comparison with CT; they are necessary for the diagnostic because they allow the lesion localization and characterization. Biopsy or tumor resections are the tools that allow the definitive diagnosis. Indolent NHL is generally considered incurable. Several regimens have been commonly used; however, the treatment has never been shown to extend the overall survival. The polychemotherapy with chop regimen is chemo regimen consisting of cyclophosphomide, doxorubicin, vincristine sulfate, prednisone represents the standard chemotherapy regimen for NHL treatment with a good outcome and few and acceptable side effects. New strategies with rituximab, a chimerical anti-CD20 immunoglobulin G1 monoclonal antibody which is a cell surface protein that occurs almost exclusively in mature B-cells, are used to improve the prognosis of these patients. The prognosis has improved in recent years owing to the development of various aggressive chemotherapeutic regimens depending on the histological type, stage, and age of each patient. Complete response is obtained in about 45%–53% of cases with long-term survival of 30%–37%.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Hennessy BT, Hanrahan EO, Daly PA. Non-Hodgkin lymphoma: An update. Lancet Oncol 2004;5:341-53.
Pileri SA, Zinzani PL, Went P, Pileri A Jr., Bendandi M. Indolent lymphoma: The pathologist's viewpoint. Ann Oncol 2004;15:12-8.
Rossi D, Gaidano G. Molecular heterogeneity of diffuse large B-cell lymphoma: Implications for disease management and prognosis. Hematology 2002;7:239-52.
Jaffe ES, Harris NL, Diebold J, Muller-Hermelink HK. World health organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. A progress report. Am J Clin Pathol 1999;111:S8-12.
Chiu BC, Weisenburger DD. An update of the epidemiology of non-Hodgkin's lymphoma. Clin Lymphoma 2003;4:161-8.
DeVita VT, Hellman S, Rosenberg SA, editors. Cancer: Principles and Practice of Oncology. 6th
ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001.
Wotherspoon AC. Gastric lymphoma of mucosa-associated lymphoid tissue and helicobacter pylori
. Annu Rev Med 1998;49:289-99.
[Figure 1], [Figure 2], [Figure 3]