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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 796-798
Leydig cell tumor : A report of two cases with unusual presentation


1 Department of Pathology, IPGME&R, 244 AJC Bose Road, Kolkata - 700 020, India
2 Park Clinic. 4 Gorky Terrace, Kolkata 17, India
3 Department of Urosurgery, IPGME&R, 244 AJC Bose Road, Kolkata - 700 020, India

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Date of Web Publication27-Oct-2010
 

   Abstract 

Leydig cell tumors (LCTs) are rare testicular tumors. They constitute 1-3% of all testicular tumors. We report two cases of LCT, one benign and the other one malignant, representing both ends of the spectrum. The case of benign LCT presented with infertility and was found to have azoospermia, and subsequently underwent orchidectomy. Histopathologic examination revealed the presence of a benign LCT. Postoperative recovery was uneventful. The sperm count improved subsequently, and a year later, he fathered a child. The case of malignant LCT was seen in another 47 year old male who presented with cough. On examination, a testicular swelling was found, and after orchidectomy, he was diagnosed to have malignant LCT on histopathologic examination.

Keywords: Infertility, leydig cell tumor, metastasis

How to cite this article:
Sengupta S, Chatterjee U, Sarkar K, Chatterjee S, Kundu A. Leydig cell tumor : A report of two cases with unusual presentation. Indian J Pathol Microbiol 2010;53:796-8

How to cite this URL:
Sengupta S, Chatterjee U, Sarkar K, Chatterjee S, Kundu A. Leydig cell tumor : A report of two cases with unusual presentation. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Dec 11];53:796-8. Available from: http://www.ijpmonline.org/text.asp?2010/53/4/796/72096



   Introduction Top


Leydig cell tumors (LCTs) are rare tumors and account for 1-3% of all testicular malignancies. Majority of these tumors are benign. Here, we report two cases of LCT, one benign and the other one malignant, representing both ends of the spectrum. The case of benign LCT presented with infertility and was found to have azoospermia. Although an increased risk of testicular cancer is associated with male infertility, [1] LCT, a benign neoplasm usually presents as a testicular mass, accompanied or preceded by hormonal changes. Azoospermia is not usually reported as a symptom of this tumor. [2] The other patient presented with symptoms related to chest metastasis.


   Case Reports Top


Case 1

A 35 year old man presented with infertility and was investigated for the underlying cause. On examination, both the testes were of normal consistency, but a mass was palpable in the left testis. Semen analysis confirmed azoospermia. Serum testosterone level, alpha feto-protein, beta-hCG levels were normal. A testicular ultrasound scan demonstrated a well-defined hypoechoic mass on the lower pole of the left testis. It measured 31×23×17mm. Both right testis and epididymis were normal. He underwent orchiectomy. After the exposure of pampiniform plexuses of both the sides, venous blood samples were taken and analyzed for testosterone level which was found to be raised in the left side. But testosterone level was normal in the right-sided venous sample. A specimen of testis 5×5×4 cm and spermatic cord with its coverings was received. On cut section, a 3 cm well-demarcated, tan colored nodule was noted in the middle. [Figure 1]a. Microscopy showed a tumor composed of large polygonal cells almost resembling hepatocytes with abundant eosinophilic, granular cytoplasm, and round, regular, vesicular nuclei with prominent nucleoli in a focally fibrotic stroma [Figure 1]b. There was no evidence of infiltrating margins, necrosis, calcification, nuclear atypia, or vascular invasion. A sprinkling of fine brown cytoplasmic pigment and crystalloids of Reinke's were noted. Immunohistochemistry showed strong vimentin positivity. [Figure 1]c. A diagnosis of benign LCT was made. Postoperative recovery was uneventful. The sperm count improved subsequently, and a year later, he fathered a child.
Figure 1 :(a) Gross photograph of the specimen of testis along with the spermatic cord showing a well-circumscribed, tan coloured nodule at the center (b)high power view of the tumor showing nests of polygonal cells with granular eosinophilic cytoplasm and round, vesicular nuclei;: (H and E, ×400); (c) immunoperoxidase stain for vimentin showing strong cytoplasmic positivity and the nucleus remains unstained (IHC, ×100)

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Case 2

We report another case of a 47 year-old man who presented with dry cough and later with hemoptysis without any endocrine symptoms. On examination, he had a swelling in right testis and chest X-ray revealed cannon ball shadows in the right lung and widening of mediastinum. Computed tomography (CT) scan of thorax was also suggestive of metastatic disease [Figure 2]a. CT scan of abdomen did not reveal evidence of para-aortic lymphadenopathy. He underwent orchidectomy subsequently. On gross examination, the specimen consisted of a testis with attached spermatic cord. There was a tan colored mass measuring 6×5.5×5 cm, replacing the normal testicular tissue with extensive areas of calcification. Microscopy showed lobules of liver like cells with abundant eosinophilic granular cytoplasm. Some cells also showed cytoplasmic clearing. There were areas of necrosis, calcification and focal atypia. The tumor had infiltrating margins and evidence of vascular invasion [Figure 2]b. The mitotic rate was less than 1/10 hpf, and on staining for MIB-1, it was less than 2/10 hpf. Both the lipochrome pigment and crystalloids of Reinke's were lacking in this case. In view of the large size, presence of necrosis, calcification, atypia, infiltrative margins and already present chest metastasis, a diagnosis of malignant LCT was made.
Figure 2 :(a) CT scan showing chest metastasis in the case of malignant LCT, (b) high power view of the malignant LCT (H and E, ×400). Lower right inset picture shows necrosis and cytoplasmic clearing, whereas, upper right inset picture shows evidence of vascular invasion in the same tumor

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   Discussion Top


LCT, although being the most common of the gonadal-stromal neoplasms, are generally rare. They constitute 1-3% of all testicular tumors. They are usually unilateral; however, 3-9% of them have been estimated to be bilateral. [2] These tumors can develop in both children and adults. In adults, they develop most frequently between 30 and 60 years of age. LCTs are usually benign, but: 10% of them have the potential to metastasize. The commonest metastatic sites are regional lymph nodes, lung, liver and bones. Men usually present with either an asymptomatic mass or with endocrine abnormalities such as gynecomastia (20-40%) or loss of libido. Azoospermia, as seen in our first case, is uncommon. [2] LCT can be non-hormone secreting or associated with autonomous secretion of steroid hormones. The most common hormones secreted are testosterone and estrogen. In our first case, azoospermia was most likely because of suppression of gonadotrophins following high level of circulating testosterone. This was supported by the fact that tumor removal resulted in complete normalization of spermatogenesis. As in the case of other endocrine tumors, like pheochromocytoma, adrenal tumors, it can be very difficult to distinguish benign LCTs from malignant ones based on histology alone. In our case 2, however, this was not a problem as the patient already came with chest metastasis. Also in this case, it appeared to follow the suggested rules of the books. The metastatic type occurs exclusively in adults. [3] and is more common in older patients with an average age of more than 40 years. [4] Our patient was 47 years old. Endocrine symptoms are rare in malignant ones, and our case 2 of malignant LCT did not show the hormonal changes as seen in case 1.

Microscopically, the LCT is made up of large polygonal cells with abundant eosinophilic and granular cytoplasm. Less frequently, the cytoplasm may be clear or vacuolated. [5] Nuclei are round and vesicular with delicate chromatin and a single prominent nucleolus. The cells usually exhibit a solid pattern of infiltration although fibrous septae may give them a pseudotubular or trabecular appearance. Crystalloids of Reinke are present in 25% of cases, but may require electron microscopic examination for their identification. On light microscopy, they appear as dense eosinophilic needle-like or rhomboid structures within the cytoplasm. LCTs are likely to be immunoreactive with inhibin and vimentin, but not cytokeratins, CD-30, Oct 3/4, or PLAP. Features of malignancy include a larger size of tumor (>5 cm), infiltrative margins, foci of necrosis, angiolymphatic invasion, nuclear atypia, mitotic count >3/10 hpf, DNA aneuploidy, and increased MIB-1 activity. [6] Even though mitotic count was low, other morphologic features like large size, necrosis, infiltrative margins, vascular invasion, and most important of all, the presence of metastasis clearly indicated malignant nature in case 2. There are cases which fall into gray zone in between benign and malignant ends of the spectrum and need to be followed up carefully for metastasis and pose difficult diagnostic problem on histology.

LCTs need to be differentiated from Leydig cell hyperplasia (LCH) and large cell calcifying Sertoli cell tumor (LCCSCT). LCH can be differentiated from LCT by the absence of discrete mass,and interstitial location of proliferating cells surrounding seminiferous tubules in a background of testicular atrophy. LCCSCT is characterized by more frequent calcification, intratubular growth, neutrophilic infiltrate, more abundant stroma and absence of Reinke's crystals.

Inguinal orchiectomy is the treatment of choice, though organ sparing surgery may be tried safely if there is follow up. No effective systemic therapeutic alternatives have been established so far.

 
   References Top

1.Negri L, Benaglia R, Fiamengo B, Pizzocaro A, Albani E, Levi Setti PE. Cancer risk in male factor-infertility. Placenta 2008;29:S178-83.  Back to cited text no. 1
    
2.Markou A, Vale J, Vadgama B, Walker M, Franks S. Testicular leydig cell tumor presenting as primary infertility. Hormones 2002;1:251-4.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Farkas LM, Szekely JG, Pusztai C, Baki M. High Frequency of Metastatic Leydig Cell Testicular Tumors. Oncology 2000;59:118-21.  Back to cited text no. 3
    
4.Rich MA, Keating MA. Leydig cell tumors and tumors associated with congenital adrenal hyperplasia. Urol Clin North Am 2000;27:519-28.   Back to cited text no. 4
[PUBMED]    
5.Billings SD, Roth LM, Ulbright TM. Microcystic Leydig cell tumors mimicking yolk sac tumor: A report of four cases. American J Surg Pathol 1999;23:546-51.  Back to cited text no. 5
    
6.Tichoo SK, Tamboli P, Warner NE, Amin MB. Testicular and paratesticular tumors. In; Weidner N, Cote RJ, Suster S, Weiss LM, editors. Modern Surgical Pathology. Philadelphia: Saunders, Elsevier Science; 2003. p. 1215-56.  Back to cited text no. 6
    

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Correspondence Address:
Subhalakshmi Sengupta
1/5 Paik Para Row, Kolkata -700 037
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72096

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