NON-Hodgkin's malignant lymphoma: a RARE CAUSE of testicular tumor
We report an anatomical-clinical observation of malignant non-Hodgkin's testicular lymphoma in a 66-year-old subject.
The diagnosis was carried on a piece of orchidectomy right.
Conventional histopathological examination and Immunohistochemical study concluded with large-cell B-phenotype diffuse lymphoma.
The extension balance showed an impairment of the Waldeyer ring and the presence of deep lumbosacral-aortic and retro-peritoneal adenopathies.
Non-Hodgkin malignant lymphomas (LMNH) revealed by testicular tumours are very rare. They account for 1% of all LMNH and 2% of LMNH occurring in humans [4]. They will gladly touch older subjects. In excess of 60 years, they represent the most common cause of testicular tumor [4].
It is usually a high-grade LMNH of malignancy.
We propose to take an update on this pathology, in the light of an anatomo-clinical observation of a subject of 66 years.
Observation
Mr. H. W, aged 66 years, with a history of pulmonary tuberculosis treated in 1971 and cataract surgery in 1998, was admitted to the Urology Department for a testicular tumor. The symptomatology was 2 months old, marked by the increase in the volume of the right purse, coupled with progressive pain and an estimated weight loss of 10 kg.
The clinical examination found a patient afebrile and in good general condition. The right testicle was increased in size, hard and painful consistency at palpation. The abdomen was supple, without palpable mass and the ganglion areas were free.
Testicular ultrasound showed a moderately swollen testicle with multiple hypoéchogènes lesions (Figure 1), these lesions were poorly constrained, contiguous in places, and not vascularized to the color Doppler examination. The étaitdiscrètement cephalic epididymis increased in size and heterogeneous appearance.
Figure 1: Ultrasound scrotal: Presence of multiple hypoéchogènes lesions poorly limited at the right testicle level.
Serum assays of tumor markers (alpha-fetoprotein, BHCG, and carcinoembryonic-embryonic antigen) were normal.
A straight orchidectomy by inguinal way was practiced. The extemporaneous exam was not performed.
The orchidectomy piece was 9 x 6 x 5 cm, with a 6 x 3 cm testicle, with a 3 x 2 cm Epididymal head with a 7 cm long cord. At the cutting, it was the seat of a firm lesion, poorly limited, 5 cm wide axis, of multinodulaire appearance and of whitish colour. She didn't show any foci of necrosis.
Histological examination showed the destruction of testicular parenchyma by a very undifferentiated malignant tumour proliferation, arranged in diffuse layers separated by fibrous tracts (Figure 2). It was made of cells of large size with little cytoplasm. The nucleus was large, irregular and provided with several nucleoli.
Figure 2: HE x 200: Malignant tumour proliferation arranged in diffuse tablecloths.
The stroma was hail and punctuated by lympho-histiocytic elements.
These elements réalisaitne on one of the cutting planes a épithélioide granuloma. Some tumor cells colonized the residual seminiferous tubes embedded in the tumor, making lymphoépithéliale-type images usually described in MALT lymphomas (Figure 3).
Figure 3: HE x 400: proliferation is made of large, irregularly large, nucleolus-sized cells. These cells colonize the residual seminiferous tubes.
Figure 4: CD 20 x 200: Tumor cells exhibit intense cytoplasmic tagging at CD20.
The tumor was infiltrating the capsule and the FBI testis. The cord and epididymis were free from tumor infiltration.
On the immunohistochemical plane, tumor cells did not express epithelial markers (Cytokeratin and EMA). They did not also express placental alkaline phosphatase (PLAP) and CD30. In contrast, they were intensely marked by lymphoid B markers (anti-CD20 and anti-CD79) (Figure 4). Anti-CD3 reacted only with non-neoplastic T lymphocytes involved in proliferation.
These histopathological and immunohistochemical data have allowed the diagnosis of diffuse lymphoma to large cells of phenotype B.
A full extension assessment involving palpation of the ganglion areas, an ENT examination with cavum biopsy, a thoraco-abdominal-pelvic scanner, a lap ultrasound, a osteo-medullary biopsy and a digestive fibroscopy, noted a Infiltration of Waldeyer ring and retro-peritoneal and lumbosacral-aortic adenopathies. Both lungs were normal in appearance.
At the end of this review, the patient was classified as stage IV according to the ANN Arbor classification modified by Musshoff.
Discussion
The first observation of testicular LMNH was reported in 1877 by Imsufficientness [9]. It is a relatively rare tumor that represents only 1 – 9% of all malignant testicular tumours [6, 15, 16]. It is the most common malignant testicular tumor of non-germ tumors in the subject aged over 50 years [15]. LMNH remains rare in young men (less than 2% of all testicular tumours) [6]. A few cases have been described in the child [1].
Testicular damage is often unilateral; It may be bilateral in 5 to 20% of cases [15]. The impairment of the contralateral testicle may be of another histological type.
immunosuppression states (AIDS, immunosuppressive therapies, tuberculosis) are favourable factors. Our patient has a history of pulmonary tuberculosis.
The generally painless increase in testicular volume is the most common sign of call. This painless character is a source of delay in diagnosis. Wescott reported the case of a patient who reportedly showed an increase in testicular volume that had evolved for 22 years before the diagnosis was carried.
Other symptoms may open the clinical picture; Slimming, anorexia, fever and altered general condition. This general symptomatology is associated with more aggressive forms of testicular lymphoma [16]. The diagnosis of testicular LMNH is done at the definitive examination after fixation and inclusion in paraffin, the extemporaneous examination being uncontributory not allowing to formally eliminate a seminoma.
As in our observation, most testicular lymphomas (90%) are B-phenotype lymphomas with large cells (high-grade malignancy) or intermediate cells [15, 19]. Some cases of T-lymphoma have been described [3, 7]. The testicular localization of Hodgkin's disease remains exceptional [4].
Testicular impairment can be isolated and primitive, but may be the first manifestation of generalized lymphoma, or manifest in the evolution of previously diagnosed lymphoma. In our observation, testicular impairment appears to be primitive given its size (5cm in diameter), the absence of superficial adenopathies, and the existence of small, shallow adenopathies.
The attainment of the Waldeyer ring is usually reported at the time of diagnosis as is the case with our patient or during the course of the disease evolution [4]. Another extra-ganglionic localization, frequently associated with testicular LMNH, is the central nervous system. This is much more common for large cell lymphomas (30% for centroblastiques, immunoblastiques, and lymphoblastic lymphomas versus 6 to 7% for other histological types) [8]. Pulmonary damage is common during the evolution of testicular LMNH. In a autopsy series of 38 patients, pulmonary disease was found in 89% of cases [14]. The Association of a skin damage to the type of papules or nodules has been reported. As a result, the discovery of testicular lymphoma systematically requires the realization of an extension balance. This assessment should include a full clinical examination, a thoraco-abdominal-pelvic scanner, a bone marrow biopsy, a gastric fibroscopy with biopsies, and an O.R. L examination for infiltration of the Waldeyer ring.
On the therapeutic level, testicular LMNH is the only lymphomateuse urogenital localization that is part of resection surgery. The orchidectomy is a rule because the testicle is not accessible to chemotherapy [4, 11]. However, even in the localized stages, the orchidectomy alone is insufficient. In fact, more than 60% of patients treated with simple orchidectomy relapse within 5 years especially at the level of the central nervous system [4]. As a result, most authors offer adjuvant chemotherapy based on anthracyclines. In young people with localized lymphoma, short-term, aggressive polychemotherapy including methotrexate or doxorubicin is recommended [17]. Pelvic radiation is increasingly being discussed. Relapse rates are greater than 50% [5, 10].
The prognosis of LMNH of the testicle is still dark with a median of survival of 12 months [8] and a survival at 5 years estimated between 15% and 50% [15, 16, 18] Despite a poly-chemotherapy. The main factors of bad prognosis recognized are: an age greater than 65 years, a significant tumor mass (> 10 cm), Ann Arbor stages III and IV, the presence of B-scalability signs, a high level of LDH (correlated to tumor mass), and the attainment of The epididymis and the spermatic cord. Young age appears to be a bad prognosis factor since it associates itself with aggressive histological forms of the lymphoblastic or Burkitt type. The bad prognosis found in our patient are the advanced age (66 years) and an IV stage of Ann Arbor.
Conclusion
The LMNH of the testicle is a rare pathology that affects especially the elderly. It is a disease of bad prognosis that dissimiles with predilection at the level of the central nervous system, the lung, the ring of Waldeyer and the skin. Its diagnosis is exclusively anatomopathologic requiring the use of immunohistochemistry in order to eliminate a séminomateuse germ tumor. The diagnosis of testicular lymphoma requires the realization of a complete extension balance in search of other locations mainly cerebral, naso-pharyngeal or pulmonary. The advent of intensive polychimiothérapies has made it possible to improve the prognosis significantly.
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