Testicular lymphoma: Atypical presentation of acute scrotal swelling

Abstract

Introduction

Primary testicular lymphoma (PTL) rarely presents as acute scrotal swelling. It is a very aggressive form of extra nodal non-Hodgkin's lymphoma. It accounts for less than 9 % of all testicular tumours. There are limited data characterizing this entity and this case report aim to add to existing literature.

Case presentation

A 40-year-old patient, with a history of a pulmonary tuberculosis declared cured, presented a scrotal swelling that set rapidly in less than a week evolving in a context of weight loss and fever. The clinical examination was tender and hard on palpation while ultrasound revealed a suspicious oval formation not taking colour in Doppler. The patient underwent a right inguinal orchidectomy due to suspicious clinical presentation.

Clinical discussion

MHNL are very rare causes of acute scrotal swelling representing approximately 1 %. It's an aggressive tumour and remains rare in young men. Diagnosis is purely histological. Standard treatment includes orchidectomy, chemotherapy with a poor prognosis.

Conclusion

PTL can reoccur years after complete remission. It's related to an expression of diffuse lymphomatosis suggesting an aggressive approach. Its management is multidisciplinary based on the tumour's stage according to Ann Arbor classification.

Highlights

• •Primary testicular lymphoma is an aggressive form of extra nodal non-Hodgkin's lymphoma.
• •It is infrequent in young patients and seldom manifests as sudden scrotal swelling.
• •MHNL of the testis is both the most common testicular malignancy in men above 60 years old.
• •Therapeutic approach depends on the tumour's stage according to the Ann Arbor classification criteria.

1. Introduction

Acute scrotal swelling is a common complaint in the emergency department. Its etiologies are well known. Malignant non-Hodgkin's lymphoma (MHNL) of the testis is a rare one. It represents 5 % of malignant testicular tumours and 1 to 2 % of cases of MHNL [1]. With a median age at diagnosis of 66–68 years, MHNL of the testis is both the most common testicular malignancy in men above 60 years old and it is the most common bilateral tumour form [2].

It is an aggressive form of cancer with a high proliferative potential. Its occurrence in young people is very rare and often has a poor prognosis.

We report an observation of an uncommon presentation of acute scrotal swelling in the emergency department, revealing testicular malignant non-Hodgkin's lymphoma (MHNL) in a young patient aged by 40. Our work has been reported in line with the SCARE Guidelines 2020 criteria [3].

2. Observation

A 40-year-old patient with a history of chronic cannabism weaned 10 years ago, and a pulmonary tuberculosis declared cured in 2017. The patient consulted the emergency department for a scrotal swelling that set in rapidly in less than a week, evolving in a context of weight loss, fever and fatigue.

On clinical examination, the right testicle was enlarged, tender and hard on palpation; the contralateral testicle was normal. The lymph nodes were free.

Due to a suspicious clinical examination, we completed with a scrotal ultrasonography that revealed two well-limited oval formations with regular contours, one hypoechoic and not taking colour in Doppler measuring 13x6mm, and the other predominantly cystic with posterior enhancement containing a few mobile echoes and echogenic portion measuring 16 × 11mm (Fig. 1).

Fig. 1.

Image showing two well-limited oval formations with regular contours on ultrasonography.

A. Predominant cystic formation with posterior enhancement containing a few mobile echoes and echogenic portion (16 × 11mm).

B. Hypoechoic formation and colourless in Doppler (13 × 6mm).

Biologically, the AFP and HCG levels were normal, while the LDH was 490 IU (2 N). Inflammatory biomarkers were also within normal range, with a CRP of 26.3 mg/l and white blood cell count at 12100 /μl, with neutrophils at 9800/μl and lymphocytes at 1300/μl. The rest of laboratory findings showed no abnormalities.

The patient underwent right inguinal orchidectomy in the emergency department (Fig. 2).

Fig. 2.

Image showing a macroscopic view of the surgical specimen.

A. Internal configuration of the surgical specimen (Nodule shown with an arrow).

B. external configuration of the surgical specimen.

Histology of the orchiectomy specimen showed diffuse large-cell B lymphoma, with an estimated Ki67 proliferation index of 80 %, and diffuse antiCD20 Ac positivity (Fig. 3).

Fig. 3.

image showing the histological aspect of the testicular lymphoma.

A. Morphological appearance of diffuse large-cell B lymphoma.

B. Ki67 proliferation index estimated at 80 %.

C. Diffuse positivity of anti-CD20.

The follow-up was based on surveillance after the patient refused chemotherapy and it was marked by a good clinical progress; an evaluation at 3 months revealed a contralateral testicle without abnormalities, a biological assessment with normal markers, and a thoraco-abdomino-pelvic CT scan without any specific findings. The patient is still under ongoing follow-up.

3. Discussion

MHNL are very rare causes of acute scrotal swelling representing approximately 1 %. They are very aggressive tumours in men over 60. Annual incidence ranges from 0.09 to 0.26 per 100,000 population. While MHNL is the most common malignant testicular tumour in the elderly, it remains rare in young men, accounting for less than 2 % of all testicular tumours [4].

Testicular lymphoma tends to be bilateral and is the most common bilateral testicular tumour [2]. The skin involvement associated with testicular lymphoma is usually associated with generalized systemic disease with a poor prognosis. The central nervous system is another extra-nodal site associated with testicular lymphoma, this involvement often being linked to a particular histological type which is lymphoblastic lymphoma and diffuse undifferentiated lymphoma [2].

Clinical symptoms are non-specific, with the patient initially feeling a painless testicular mass. On examination, the testicle is well differentiated from the epididymis (CHEVASSU+), with a sensation of testicular heaviness. General signs may be associated, such as weight loss, fever, and anorexia. In patients with systemic symptoms, their disease was often aggressive, linked mainly to diffuse lymphomatosis [4].

Patients with testicular MHNL must benefit from a full medical history, including a search for systemic symptoms (fever, weight loss, etc.), and a complete physical examination including the contralateral testicle, the search of supra and subdiaphragmatic lymphadenopathies, hepatosplenomegaly, and a skin examination.

A standard biological test includes serum AFP (alpha-fetoprotein), total HCG (human chorionic gonadotropin) and LDH (lactate dehydrogenase) to assess tumour volume. Because of the risk of neurological damage, lumbar puncture with cytological analysis of the fluid is recommended [1].

Radiological exams encompass a testicular ultrasound to confirm the diagnosis, revealing either focal or diffuse areas of hypoechogenicity with hypervascularity in an enlarged testis. Additionally, magnetic resonance imaging provides further evaluation of both testes, paratesticular spaces, and the spermatic cord [1]. A thoraco-abdomino-pelvic CT scan is essential to ascertain the tumour's extent and to discern whether it is primary or secondary.

About therapeutic management, testicular MHNL is the only urogenital lymphomatosis requiring surgical excision. The inguinal approach of orchidectomy, considered as the gold standard, is an essential component of diagnosis and treatment [5], due to the testicle is an organ that is not easily accessible to chemotherapy, and it is a frequent site of relapse. Successive lymphoma involvement of the opposite testicle may occur months or years after orchidectomy and might be of a different histological subtype.

After that, the therapeutic approach depends on the tumour's stage according to the Ann Arbor classification criteria. The vast majority (70 %–80 %) are diagnosed at a localized stage (stage I -II). Advanced stages (stage III-IV) are very rare [6].

For localized stages, orchidectomy alone is not sufficient. Indeed, 60 % of patients recur within the 5 years, most often in the central nervous system. Most authors opt for adjuvant chemotherapy with or without radiotherapy. For patients who cannot tolerate or refuse chemotherapy, radiotherapy of the pelvic, inguinal, and para-aortic lymph nodes should be performed, as a result 40–50 % of patients remain free of disease at 2 years. Central nervous system chemoprophylaxis and scrotal radiotherapy are recommended for these stages [7].

In advanced stages (III-IV), the reference treatment remains anthracycline chemotherapy. Intrathecal chemotherapy is recommended at all stages of the disease to prevent CNS relapse [7]. The place of scrotal radiotherapy in these stages is controversial.

Analysis of cases reported in the literature shows a poor prognosis of testicular lymphoma, particularly in diffuse forms (stages III and IV), despite chemotherapy with a very low survival rate [7].

4. Conclusion

MHNL of the testis in young men are rare and have a poor prognosis. They are often the expression of diffuse lymphomatosis. This extensive nature of the disease explains the therapeutic failures observed. An aggressive approach is therefore advisable.

Management of this tumour is multidisciplinary based on its stage according to the Ann Arbor classification.

Consent

A consent was obtained from the patient to publish this case report and accompanying images.

Ethical approval

Ethical approval is not applicable. The case report is not containing any personal information.

Funding

No funding or grant support.

Author contribution

Jihad LAKSSIR, Adam ELABOUDI: performed surgery, paper writing and editing.

Ahmed IBRAHIMI, Hachem El-SAYEGH, Yassine NOUINI: literature review, Supervision.

Jihad LAKSSIR, Omar BELLOUKI: Manuscript editing, picture editing.

Guarantor

Jihad LAKSSIR M.D., Urology A Department, Ibn Sina University Hospital Center,

Morocco.

Email: j.lakssir@gmail.com

Registration of research studies

Not applicable.

Conflict of interest statement

The authors declare that they have no competing interests relevant to the content of this article.