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Alaqqad, Kannan, Ram, Elshamsy, Elhefni, and Khan: A case of diffuse large B-cell lymphoma presenting in a patient with a family history of other types of cancers

Introduction

Lymphoma is one of the most common cancers.1 The two main forms of lymphoma are Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL). There are two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). Lymphoma can spread to any organ and form tumours.1

There are at least 61 types of NHL. B-cell lymphomas develop from abnormal B-lymphocytes and account for 85% of all NHLs. T-cell lymphomas develop from abnormal T-lymphocytes and account for the remaining 15% of all NHLs. NHLs may also be further classified as indolent (slow-growing) or aggressive (fast-growing).2

Common signs and symptoms of NHL include swelling of the lymph nodes (which is often but not always painless), fever, night sweats, unexplained weight loss and lack of energy. Although most people with these complaints will not have NHL, anyone with persistent symptoms should be seen by a physician to exclude lymphoma.

Factors that may increase an individual’s risk of developing NHL include:3

  • family history

  • age

  • gender (men are more likely to develop NHL than women)

  • bacterial infection: mucosa-associated lymphoid tissue, lymphoma and Helicobacter pylori

  • viruses: such as Epstein–Barr virus

  • immune deficiency and autoimmune disorders

  • previous organ transplant

  • previous cancer treatment

  • chemical exposures.

Treatment options for NHL include:

  • radiation therapy directed at the area where cancer is found and adjacent lymph nodes

  • watchful waiting

  • chemotherapy with radiation therapy

  • monoclonal antibody therapy with or without chemotherapy.4

T-cell/histiocyte-rich large B-cell lymphoma

T-cell/histiocyte-rich large B-cell lymphoma is a rare subtype of diffuse large B-cell lymphoma (DLBCL) characterized by abundant non-neoplastic T-cells and histiocytes that may obscure tumour cells, which was observed in this case.5

Case presentation

A young man, 31 years old, was referred to our clinic for evaluation of a lump in the left inguinal region, which had been present for 6 months. The patient reported that swelling had gradually increased in size and reported that it was painless and not associated with any symptoms. There was no history suggestive of malignancy.

The patient had undergone an uneventful open appendectomy 1 year previously. Histopathology confirmed an inflamed appendix. The patient had no history of chronic illness. He was married and denied any history of sexually transmitted disease. There was no history of travel-related disease.

One year previously, the patient’s father had died with a malignant tumour in the lung. In addition, the patient’s grandmother had been diagnosed with carcinoma of the gall bladder and later died.

On examination, the patient’s physical appearance was good and he was not pale or jaundiced. His cervical axillary lymph nodes were not enlarged. Abdominal examination revealed a right lower quadrant scar from a previous appendectomy. His left inguinal region showed a well-defined spherical lump extending obliquely parallel to the inguinal ligaments; the skin over the lump was normal. This lump was around 12 cm in diameter, and was not tender or attached to the skin. It had a firm to hard consistency and was slightly fixed. There was no coughing impulse and light transillumination was negative. Scrotum and perineum were normal. Small palpable lymph nodes were observed in the right inguinal area. His lower limbs showed no abnormal growth, ulceration or chronic wounds and systemic examination was unremarkable.

We carried out investigations with the aim of identifying the common causes of chronic enlargement of lymph nodes; colonic tumour markers were negative. The patient underwent abdominal and pelvic ultrasonography, but the results were unremarkable. However, scrotal ultrasonography revealed features of chronic epididymo-orchitis. Moreover, a large soft-tissue component, composed of multiple separate soft tissues, was noted along the left inguinal canal, and groups of femoral lymph nodes were interconnected with the pelvic lymph nodes along the internal iliac artery on the same side. The involved lymph nodes were solid and showed a peculiar homogeneous reduced echogenicity with posterior acoustic enhancement due to sonic transmission through well-defined outlines and peculiarly of normal echogenic hila. The degree of roundness was assessed from the longest to shortest axis ratio (L/S). However, a significant increase in vascularity was apparent through the hila of the lymph nodes. Interpretation of the findings was based on the fact that in patients with inflammatory diseases the process is diffuse and the nodal shape is more likely to be preserved and the hila are echogenic, whereas, in malignant states, hilar invasion via the afferent lymphatics, penetration of the capsule and subcapsular sinus infiltration are noted. Metastases result in peripheral involvement of the nodes, causing cortical enlargement, which may be eccentric; therefore, a cortical bulge often precedes generalized cortical enlargement and distortion or destruction of the intranodal architecture, with loss of the hilum. Figure 1 shows lymph nodes detected by ultrasound.

FIGURE 1

Inguinal lymphadenopathy by superficial ultrasound probe is shown in this case. Image A shows the dimension while B shows the vascularity (vessels appear in blue).

8-4-2-fig1a.jpg8-4-2-fig1b.jpg

Computerized tomography (CT) of the abdomen and pelvis with oral contrast was carried out before and after intravenous contrast administration. The outcome revealed the same findings regarding the above-mentioned masses. The lesions showed significant homogeneous contrast enhancement with no evidence of central breakdown, matting or calcifications. The masses varied in size (30–60 mm in diameter); however, other similar but smaller soft-tissue lesions were noted distally along the left femoral veins, measuring 10–13 mm in diameter. Further smaller lesions were seen proximally, arranged along the left common iliac vein and on the left para-aortic and retroaortic groups of lymph nodes, which measured 11–15 mm in diameter and showed similar attenuation and enhancement patterns. The report suggested a diagnosis of lymphadenopathy along a specific vascular territory involving sequential groups of lymph nodes. The aetiology suggested localized lymphadenopathy rather than a systemic disorder or malignancy. It ought to be mentioned that detection of the disease in normal-sized lymph nodes is not possible and, even with enlarged lymph nodes, it is not possible to differentiate between reactive hyperplasia and lymphoma by cross-sectional imaging. Therefore, tissue biopsy was recommended for further assessment. CT images showing the lymph nodes are provided in Figure 2.

FIGURE 2

CT images of the enlarged inguinal lymph nodes are shown in A and B after intravenous contrast administration, which shows the high degree of definition and homogeneous enhancement pattern of these nodes associated with stranding of the subcutaneous tissues.

8-4-2-fig2a.jpg8-4-2-fig2b.jpg

The patient was prepared for excisional biopsy under general anaesthesia. The excised lymph node is shown in Figure 3. This lymph node was sent for histopathology.

FIGURE 3

Excised inguinal lymph node.

8-4-2-fig3.jpg

Discussion

The mechanisms underling NHL and a family history of other cancers are still uncertain and not well understood,6 so in this case there could be a relation between NHL and a family history of cancers. This young man had only a family history of cancer without other risk factors related to his age, previous cancer and exposure to chemicals or even viral infection, all of which can be associated with malignancy.

Of particular interest is that the patient presented with enlarged painless inguinal lymph nodes but with no night sweats, fever or weight loss. His father died from small cell lung cancer, while his grandmother died from carcinoma of gall bladder several years previously. In addition, three different types of cancers were found among members of the patient’s extended family.

As regards the radiological findings, and according to the World Health Organization, a maximum short axis of 10 mm is considered normal in the appendicular regions, and 6 mm is the maximum upper limit in the pelvis. This proves that the patient had lymphadenopathy. The features of these masses are shown in Figure 1.

Sonography features that have been used to characterize lymph nodes as benign or malignant include size, shape, presence or absence of an echogenic hilum, cortical morphology, echogenicity, nodal border, calcification, cystic change, necrosis and vascular patterns. No single morphological feature specific for malignancy or benignity has been reported, although there are combinations of sonographic features that are suggestive of benignity or malignancy, and further assessment by CT of the neck, chest, abdomen and pelvis may help to determine whether a biopsy should be performed. The results of our patient’s biopsy revealed atypical lymphoproliferative disorder but it was not possible to determine if the resected tissue was benign or malignant.7 Thus, the diagnosis was confirmed with the help of immune markers. However, in many types of tumours, occurring in numerous body locations, the pattern of immune markers mimics that found in lymphoma.7 Doctors are currently trying to prove the relation between types of lymphoma and other cancers such as skin cancer, breast cancers and thyroid cancer.810

Specifically, T-cell/histiocyte-rich large B-cell lymphoma is a rare subtype of lymphoma accounting for less than 2% of all lymphomas,11 and was found in this patient.

The correlation between this rare type of lymphoma and family history of cancer is not clear.

Conclusion

Lumps in the inguinal regions in adults should be taken seriously and fully investigated even in the absence of lymphomatous constitutional symptoms. We can conclude that there may be a relation between lymphoma and a family history of other types of cancers.

Acknowledgements

There are many people to thank, but the first and foremost is the patient, who was so cooperative. I hope he will be cured soon. In addition, I would like to thank my colleagues for their invaluable support and assistance.

References

1. 

Yale School of Medicine. Hematology. URL: http://medicine.yale.edu/cancer/patient/programs/hematology/index.aspx (accessed November 2014).

2. 

Lymphoma Research Foundation. Non-Hodgkin lymphoma (NHL). URL: www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300139&gclid=CJKmk6WTpcECFSXKtAodGCwAOw#Symptoms (accessed November 2014).

3. 

American Society of Clinical Oncology. Lymphoma – non-Hodgkin: risk factors. Cancer.Net; 2014. URL: www.cancer.net/cancer-types/lymphoma-non-hodgkin/risk-factors (accessed November 2014).

4. 

National Cancer Institute. Treatment option for NHL URL: www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient/page5 (accessed November 2014).

5. 

Pittaluga S, Jaffe ES. T-cell/histiocyte-rich large B-cell lymphoma. Haematologica 2010; 95:352–6. http://dx.doi.org/10.3324/haematol.2009.016931

6. 

Chatterjee N, Hartge P, Cerhan JR, et al. Risk of non-Hodgkin’s lymphoma and family history of lymphatic, hematologic, and other cancers. Cancer Epidemiol Biomarkers Prev 2004; 13:1415–21.

7. 

Waduge RN, Ratnatunga NVI, Ramadasa S. Unusual histological features of non Hodgkin’s lymphoma: a clinicopathological and immunohistochemical study. J Diagn Pathol 2002–2003; 1:17–21.

8. 

Hussein MRA. Atypical lymphoid proliferations. Expert Rev Hematol 2013; 6:139–53. http://dx.doi.org/10.1586/ehm.13.4

9. 

Adami J, Frisch M, Yuen J, Glimelius B, Melbye M. Evidence of an association between non-Hodgkin’s lymphoma and skin cancer. BMJ 1995; 310:1491–5. http://dx.doi.org/10.1136/bmj.310.6993.1491

10. 

Wiernik PH, Hu X, Ratech H, et al. Non-Hodgkin’s lymphoma in women with breast cancer. Cancer J 2000; 6:336–42.

11. 

New York Thyroid Center. Thyroid lymphoma. Columbia University, Department of Surgery; 2015. URL: http://columbiathyroidcenter.org/thyroid_lymphoma.html (accessed November 2014).




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