|Year : 2017 | Volume
| Issue : 3 | Page : 181-183
Leydig cell tumor of testis in a child: An uncommon presentation
Madhumita Mukhopadhyay1, Chhanda Das1, Sucharita Sarkar1, Biswanath Mukhopadhyay2, Bedabrata Mukhopadhyay3, Rishavdeb Patra4
1 Department of Pathology, IPGME and R, Kolkata, West Bengal, India
2 Department of Paediatric Surgery, Apollo Gleneagles Hospital, Kolkata, West Bengal, India
3 Department of Biochemistry, IMS, BHU, Varanasi, Uttar Pradesh, India
4 Department of Pediatric Surgery, IPGME and R, Kolkata, India
|Date of Web Publication||8-Jun-2017|
31 Eastern Park, First Road, Santoshpur, Kolkata - - 700 075, West Bengal
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Leydig cell tumors (LCTs) are rare testicular tumors. Incidence is 1%–3% of all testicular neoplasms, bilateral in 10%. They are frequently hormonally active, leading to feminizing or virilizing syndromes. LCTs can be either pure or mixed with germ cell tumors or other sex cord-stromal tumors. Here, we are reporting a benign pure LCT in a 6-year-old boy presented with pseudopuberty.
Keywords: Calretinin, pseudopuberty, testicular tumor
|How to cite this article:|
Mukhopadhyay M, Das C, Sarkar S, Mukhopadhyay B, Mukhopadhyay B, Patra R. Leydig cell tumor of testis in a child: An uncommon presentation. J Indian Assoc Pediatr Surg 2017;22:181-3
|How to cite this URL:|
Mukhopadhyay M, Das C, Sarkar S, Mukhopadhyay B, Mukhopadhyay B, Patra R. Leydig cell tumor of testis in a child: An uncommon presentation. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2020 Feb 28];22:181-3. Available from: http://www.jiaps.com/text.asp?2017/22/3/181/207641
| Introduction|| |
Leydig cell tumors (LCTs) are rare testicular tumors arising from male gonadal interstitium  and most common type of testicular sex cord-stromal tumor., Adult male between 20- and 60-year age group usually affected , prepubertal (most often between 5 and 10 years of age) children are also affected in 20% of cases. Testicular swelling, decreased libido (20%), and gynecomastia (15%) are common symptoms in adults, but in children only a few cases had been reported and that are associated with pseudopuberty.
| Case Report|| |
A 6-year-old boy presented with precocious puberty [Figure 1]. On isotope scan, bone age was >12 and <14 years. Ultrasound reveals a heterogeneous echogenic space-occupying lesions involving whole left testis with many micro- and macrocalcification and increased vascularity. The volume of left testis was 12 cc. Hormonal assay showed luteinizing hormone (LH) levels was <0.07 U; normal human chorionic gonadotropin level <1 mIU/ml; alpha-fetoprotein (AFP) level 0.97 IU/ml; serum testosterone 17.9 nmol/L; and serum cortisol, adrenocorticotropic hormone, and 17 OH progesterone level were 8.86 μ/dl, 37.9 pg/ml, and 31.56 ng/ml, respectively. All levels are within normal limits except testosterone levels are raised. Computed tomography abdomen was normal. The patient underwent orchiectomy. Grossly, testis with scrotum was 5.5 cm × 3.5 cm × 2.5 cm, testis was 4 cm × 3 cm × 2 cm, and attached spermatic cord was 5.5 cm in length. Cut section shows lobulated, yellow well-circumscribed mass [Figure 2]. On microscopy, sections show polygonal cells with abundant eosinophilic cytoplasm and prominent nucleoli arranged in sheets and nodular pattern [Figure 3]. Pleomorphism present at places. Immunohistochemical staining with calretinin done, showed positive staining [Figure 4]. Diagnosis of benign LCT was made from clinical, hormonal, pathological, and immunohistochemical study.
|Figure 3: Polygonal cells with abundant eosinophilic cytoplasm, prominent nucleoli (×400)|
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| Discussion|| |
Leydig cells are named after Franz Leydig. They are interstitial cells located between the seminiferous tubules. They are involved in the development of secondary male characteristics and maintenance of spermatogenesis as they produce testosterone when stimulated by LH. The origin of tumor is still unknown. The excess LH may lead to disruption of the hypothalamic-pituitary testicular axis leading to excessive stimulation of Leydig cells. Rare cases are reported associated with cryptorchidism and Klinefelter Syndrome  although cryptorchidism is not considered as a risk factor. Grossly, the tumor is small (3–5 cm), sharply circumscribed, solid mass within the testicle. The color of the tumor is characteristic ranging from brown to golden brown to gray-white (depending on the lipid or lipofuscin content of the tumor). Large size (>5 cm), infiltrative margins, areas of hemorrhage, and necrosis extending beyond testicular parenchyma suggest malignancy. Microscopically, large polygonal tumor Cells, abundant eosinophilic granular cytoplasm with indistinct border is most commonly arranged in nests or sheets, separated by delicate fibrovascular septa. Nucleus is round to oval sometimes with a prominent nucleolus. Other cell types can be found small cells with scant, densely eosinophilic cytoplasm, and grooved nuclei and spindle cells. Some cells may have pale cytoplasm due to the presence of glycogen. Abundant lipofuscin (also called lipochrome) pigments which appear golden yellow to brown cytoplasmic (mainly perinuclear) granules on hematoxylin and eosin stain. It also can be demonstrated by periodic acid–Schiff stain with or without diastase. Reinke crystals are pale-staining, refractile, cylindrical, rectangular, or rhomboid intracytoplasmic and intranuclear structures (or inclusions) arranged in a linear fashion. Presence of reinke crystals has no significance. Rare cases of LCT with adipocyte differentiation, ossification, calcification, fibrous or myxoid stroma, or microcystic growth pattern have also been reported. LCT should be differentiated from Leydig cell hyperplasia, granular cell tumor, malakoplakia, the testicular nodules of adrenogenital syndrome, and large cell calcifying Sertoli cell tumor which commonly has a retiform pattern. Malignancy is suspected in the presence of a large tumor size (>5.7 cm), nuclear atypia, a mitotic count of >3/10 HPF, foci of necrosis, infiltrative margins, angiolymphatic invasion, an increased expression of Ki67/MIB-1 and p53, and DNA aneuploidy. LCTs show diffuse cytoplasmic positivity for calretinin, inhibin, vimentin, Melan-A, and negative immunostaining with cytokeratin AFP. In case of malignant and borderline cases (positive) of LCT, bcl-2 could be helpful. Radical inguinal orchidectomy is the classical treatment, but now testis-sparing surgery by tumor enucleation with clear margins is considered treatment of choice, especially in young patients, to maintain their fertility. For malignant LCTs, orchiectomy with retroperitoneal lymphadenectomy is required as metastasis most commonly involves retroperitoneal nodes other than liver (45%), lungs (40%), and bone (25%). Survival time is reduced to approximately 3 years after surgery. That is in contrast for benign LCTs. Prognosis is good with subsidence of clinical and hormonal manifestations 90% of patients following orchidectomy. Sönmez et al. and Ilkhanizadeh et al. reported LCT in adult male, whereas Singh and Chauhan et al. reported a case in a 13-year-old boy, but none found this entity like us in a 6-year-boy.
| Conclusion|| |
LCTs are uncommon neoplasm arising from gonadal stroma. It can be early detected by self-examination of testicles. Early detection and management of these tumors is necessary to preserve the reproductive capacity with long-term follow-up for recurrence or metastasis.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]