Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 6  |  Page : 819-821  

Epididymal lymphangioma: An unusual cause for retrotesticular mass in an adult


1 Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India
2 Department of Urology, Kasturba Medical College, Manipal University, Mangalore, Karnataka, India

Date of Web Publication19-Nov-2015

Correspondence Address:
Jessica Minal
Department of Pathology, Kasturba Medical College, Manipal University, Light House Hill Road, Mangalore - 575 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.169941

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  Abstract 

Lymphangioma is a congenital benign tumor caused by developmental malformations of the lymphatic system. These lesions are generally recognized at birth or in early childhood. The most common location is the head and neck region. Lymphangioma of the epididymis is very unusual as its occurrence in the adult age group. The correct diagnosis is essential for proper management. We report an unusual case of epididymal lymphangioma in a 46-year-old male presenting as a painful retrotesticular mass.

Keywords: Adult, epididymis, lymphangioma, retrotesticular swelling


How to cite this article:
Kini H, Minal J, Prabhu LG, Suresh PK, Basavaiah SH. Epididymal lymphangioma: An unusual cause for retrotesticular mass in an adult. Med J DY Patil Univ 2015;8:819-21

How to cite this URL:
Kini H, Minal J, Prabhu LG, Suresh PK, Basavaiah SH. Epididymal lymphangioma: An unusual cause for retrotesticular mass in an adult. Med J DY Patil Univ [serial online] 2015 [cited 2020 Nov 24];8:819-21. Available from: https://www.mjdrdypu.org/text.asp?2015/8/6/819/169941


  Introduction Top


Lymphangioma is a benign tumor caused by congenital malformations of the lymphatic system. [1] It is usually recognized at birth, in childhood or in early adolescence and occasionally in adults. It presents as a slowly enlarging soft mass. The most common location of this tumor is head, neck and axilla. Occasionally, it is seen in the mediastinum and retroperitoneum. Lymphangioma occurring in the perineum and scrotum is very rare. [2],[3] Approximately, 18 cases of scrotal lymphangiomas in adults have been reported in the literature till date to the best of our knowledge. It presents with nonspecific symptoms and should be considered in the differential diagnosis of paratesticular and retrotesticular swellings to avoid misdiagnosis, improper treatment and recurrence. [4]

We report an unusual case of lymphangioma of the epididymis in a 46-year-old male that presented as a retrotesticular mass. This case report is an unusual presentation of lymphangioma at a rare site and in rare age.


  Case Report Top


A 46-year-old male presented to the urology clinic with pain in both the testis since 5 years. The pain was dull aching in nature. He also noticed a swelling in the right testis since 1-year. Clinical examination revealed a palpable painful mass in the right retrotesticular region. The mass was felt separately from the testis, and the overlying skin was free from the lesion. Bilaterally, the testes were normal but the epididymis was firm to hard. Any prior history of trauma or infection was absent.

Ultrasonogram of the scrotum with Doppler suggested bilateral epididymo-orchitis with focal collection (13 mm × 7 mm) adjacent to right spermatic cord, minimal left hydrocele and left Grade I varicocele. A provisional clinical diagnosis of tuberculous epididymitis was given, and antitubercular therapy was suggested empirically. However, the treatment was not started as the subsequent urine microscopy and culture were negative for acid fast bacilli. The right sided retrotesticular lesion was surgically excised to determine the exact nature of the lesion and to rule out a possible scrotal neoplasm.

The specimen consisted of a grey white mass measuring 4 cm × 4 cm × 2 cm with the cut section showing a cyst lined by thickened shaggy wall and grey white fibrotic tissue with scant pale white mucoid fluid. The tunica vaginalis appeared edematous [Figure 1]. Microscopy revealed epididymis with adjacent soft tissue showing a meshwork of interconnecting ectatic dilated to irregular spaces lined by a single layer of flattened endothelial cells. The endothelial lining showed absence of atypia, pleomorphism or hyperchromasia [Figure 2]. The lumen was devoid of red blood cells (RBCs). The spaces extended up to the proximal spermatic cord. The intervening stroma consisted of fibrocollagenous tissue, bundles of smooth muscle cells, few blood vessels and lymphocytic infiltrate.
Figure 1: Cyst lined by thickened shaggy wall and gray white fibrotic tissue and edematous tunica vaginalis

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Figure 2: Meshwork of interconnecting ectatic vessels lined by a single layer of flattened endothelial cells. The spaces are devoid of red blood cells. There is the absence of atypia and pleomorphism

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The postoperative period was uneventful, and the patient was followed-up for a period of 6 months in outpatient clinic without any significant complaints.


  Discussion Top


Lymphangioma is benign lymphatic malformations of the skin and the subcutis usually presenting at birth or in childhood and accounts for approximately 26% of benign vascular tumors in children. However, it is quite uncommon in adults. These present as painless mass lesions in the head and neck region (95%). [1] Uncommonly, it arises in the scrotum, retroperitoneum or abdominal wall. Lymphangioma of the epididymis in an adult may arise as a true primary neoplasm or secondary to lymphatic obstruction associated with previous surgery or trauma. [2] However, this distinction has no practical utility as all of these tumors are benign and require surgery. [5] Lymphangioma was first described by Whimster in 1976. He proposed that it arises during embryonic development and is composed of subcutaneous dilated lymphatics. It is formed as the primitive lymphatic spaces are not connected with the drainage system and hence are unable to drain the lymph from surrounding tissue and as such dilate to form a swelling. [6]

The vascular tumors are generally slow growing and nonsymptomatic but may present with acute onset of pain due to associated bleeding and infection. Physical examination suggests an unencapsulated mass apparently separate from the scrotal content. Microscopically, the cysts or the spaces are lined by flattened bland endothelial cells and are separated by variable amount of stroma. The spaces are devoid of RBCs, which assist in differentiating these vascular neoplasms from hemangiomas. Though benign, it is unencapsulated and frequently has an infiltrative nature, which may complicate surgical excision. [3]

The differential diagnosis for extratesticular scrotal masses includes predominantly benign conditions like hydrocele, spermatocele, varicocele, abscess, hematoma, sperm granuloma, tuberculosis, adenomatoid tumor and lipoma. Metastasis accounts for most of the malignant lesions. Lymphangioma is a rare but important cause of extratesticular, intrascrotal mass. [3] It is important to recognize this entity as one of the differential diagnoses of testicular and paratesticular tumors for the correct clinical management and curative approach. [4]

It is difficult to diagnose this lesion in the inguino-genital regions because of the nonspecific nature of complaints in the majority of the cases, and the final diagnosis is usually elusive until the surgery is performed. [7] A study reported seven cases of scrotal cystic lymphangioma over a 10-year period, all of which were misdiagnosed preoperatively and four out of the seven cases recurred because of incomplete excision. [8] Ultrasonography plays an important role in establishing the diagnosis of lymphangioma at these uncommon sites. These lesions are typically described as lobulated, multiseptate or multicystic masses. The treatment of choice is complete surgical excision. [3] Recurrence is seen within 3 months in 25-50% of patients due to incomplete excision of the tumor. [6],[9] Other modalities of treatment include a wide variety of options such as surface abrasion by X-ray therapy, radiotherapy, argon laser, 900 nm diode laser, pulsed dye laser and sclerotherapy with observation. [6],[10]

Lymphangioma is uncommon hamartomatous lymphatic malformations. Its localization in the genital region and particularly in the epididymis is extremely rare. It usually presents at birth or early childhood and its occurrence in adults is unusual. We report a case of lymphangioma occurring at 46 years of age in retrotesticular region. A high degree of suspicion is required, and lymphangioma should be considered in the differential diagnosis of an extatesticular multiloculated lesion. Correct diagnosis is mandatory and avoids mismanagement and calls for complete surgical excision to prevent recurrences.

 
  References Top

1.
Jamal YS, Rahman A, Halim A, Moshref SS, Kurdi MO, Sandugji HI. Cystic lymphangioma of spermatic cord: A case report and literature review. JKAU Med Sci 2009; 16:103-11.  Back to cited text no. 1
    
2.
Pai KK, Roy D. Lymphangioma of epididymis: An extremely rare cause of scrotal swelling. Indian J Urol 2006;22:275-6.  Back to cited text no. 2
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3.
Weidman ER, Cendron M, Schned AR, Harris RD. Scrotal lymphangioma: An uncommon cause for a scrotal mass. J Ultrasound Med 2002;21:669-72.  Back to cited text no. 3
    
4.
Rastogi R, Meena GL, Kumar R, Rastogi V. Cystic lymphangioma scroti: A common tumor at a rare location. Saudi J Kidney Dis Transpl 2010;21:1132-4.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Postius J, Manzano C, Concepción T, Castro D, Gutierrez P, Bañares F. Epididymal lymphangioma. J Urol 2000;163:550-1.  Back to cited text no. 5
    
6.
Haroon S, Hasan SH. Lymphangioma circumscriptum in the scrotum: A case report. J Med Case Rep 2012;6:233.  Back to cited text no. 6
    
7.
Gunn SW. Lymphangioma of the epididymis. Can Med Assoc J 1959;81:30-2.  Back to cited text no. 7
    
8.
Hurwitz RS, Shapiro E, Hulbert WC, Diamond DA, Casale AJ, Rink RC. Scrotal cystic lymphangioma: The misdiagnosed scrotal mass. J Urol 1997;158:1182-5.  Back to cited text no. 8
    
9.
Olabanji J, Oladele A, Famurewa O, Adejuyigbe O, Ademola S. Retroperitoneal and genital lymphangioma therapeutic challenges in a developing country. Libyan J Med 2009;4:44-5.  Back to cited text no. 9
    
10.
Mohanty S, Arora VK, Gandhi V, Singal A, Baruah MC. Lymphangioma circumscriptum of scrotum of late onset. Indian J Dermatol Venereol Leprol 1998;64:289-90.  Back to cited text no. 10
[PUBMED]  Medknow Journal  


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