HISTOPATHOLOGY SECTION - CASE REPORT
Year : 2008 | Volume
: 51 | Issue : 1 | Page : 70--71
Testicular retiform Sertoli cell tumor: A problem in histopathologic diagnosis
K Mardi, J Sharma
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
12 A, Type V Quarters GAD colony, Kasumpti, Shimla, Himachal Pradesh
A 9-year-old boy who presented with a left scrotal swelling was subsequently diagnosed as retiform sertoli cell tumor of testis which consisted entirely of retiform pattern. The case is reported in view of its rarity in this age group as well as its unique histologic appearance.
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Mardi K, Sharma J. Testicular retiform Sertoli cell tumor: A problem in histopathologic diagnosis.Indian J Pathol Microbiol 2008;51:70-71
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Mardi K, Sharma J. Testicular retiform Sertoli cell tumor: A problem in histopathologic diagnosis. Indian J Pathol Microbiol [serial online] 2008 [cited 2022 Jun 30 ];51:70-71
Available from: https://www.ijpmonline.org/text.asp?2008/51/1/70/40404
Sertoli cell tumor of the testis is extremely rare in the pediatric population.  Despite their rarity, Sertoli cell variants of sex cord-stromal tumors have generated keen interest because of their variable histologic appearance. We report one such rare sertoli cell tumor in a 9-year-old boy which almost entirely comprised of retiform pattern.
A 9-year-old boy presented to the surgical out patient department with complaints of painless left scrotal swelling. Ultrasound showed a low-echoic mass in the left testis. The clinical impression was that of a testicular tumor and left orchiectomy was conducted.
It was a well-circumscribed tumor measuring 4 x 3.2 x 1.5 cm 3 in the left testis. Cut section of the tumor was yellowish-white with minute cystic areas. Microscopic examination revealed a tumor composed of network of irregularly branching, elongated, narrow slit-like tubules, and cysts. There were delicate papillae projecting in to the lumen of the cysts [Figure 1]. In focal areas, the papillae were small rounded with hyalinized cores as well as large bulbous with edematous cores. These papillae as well as hollow tubules were lined by epithelial cells exhibiting stratification and atypia. The stroma was loose and edematous in some areas and hyalinized in other areas. The case was diagnosed as retiform Sertoli cell tumor of the left testis.
Because of the remarkable diversity of patterns and cell types encountered in the gonads, evaluating them is one of the most interesting challenges that faces the diagnostic pathologist. Knowledge of what tumors have the patterns and cell types observed is crucial in arriving at the correct diagnosis. Sertoli cell tumors occur in both gonads being more common in the testis where they usually exhibit a lobular pattern of hollow or solid tubules. Exceptionally, they have a retiform morphology  which is characterized by an irregular network of elongated, often slit-like tubules and cysts that often contain papillae and resembled the rete testis. A lack of awareness of this pattern, which has not been described adequately in the literature, results in frequent initial misinterpretation of these tumors as neoplasms of other types,  including endodermal sinus tumor which are more malignant neoplasms requiring different therapy.  This further underlines the importance of recognizing this histopathologic entity.
Retiform pattern is only encountered in Sertoli cell tumors that are otherwise intermediate, poorly differentiated, or heterologus.  But retiform Sertoli cell tumor is entirely composed of tubular structures arranged in a pattern resembling that of the rete testis. ,, On gross examination, Sertoli cell tumors with retiform components are more often cystic and may contain large, edematous papillae.  Characteristic histologic features include a network of irregularly branching, elongated, narrow, often slit-like tubules, and cysts in to which papillae and polypoidal structures may project. These tubules and cysts may contain eosinophilic secretions. The papillae and polyps are of three types. Most commonly they are small and rounded or blunt, often with hyalinized cores. Sometimes they are large and bulbous containing edematous cores. Less commonly they are delicate and branch extensively.
Guo et al.  have described the pelvic washing cytomorphology of an ovarian Sertoli-Leydig-cell tumor with a retiform pattern. The cytologic features in their case were tight tissue fragments composed of small, relatively uniform cells with scanty cytoplasm and small rounded or blunt papillary fragments with hyalinized cores lined with small, mildly atypical cuboidal cells. The main differential diagnosis with the present case was with the yolk sac tumor.
Retiform sex cord-stromal tumors must be mainly differentiated from yolk sac tumors of the testis. The papillae of the yolk sac tumor are usually lined by poorly differentiated cells. The presence of Schiller-Duval bodies and positive immunohistochemical stain for α-fetoprotein also support the diagnosis of yolk sac tumor.
Immunohistochemistry is useful in distinguishing retiform Sertoli-Leydig cell tumors from other tumors that they may resemble. Mooney et al.  immunohistochemically evaluated eight Sertoli cell tumors with retiform differentiation. The result showed a more intense positivity for keratin and vimentin in the five cases studied, and for inhibin in one case in the retiform areas. Thus inclusion of inhibin is essential in a panel of antibodies to evaluate these tumors.
It is difficult at this stage to comment about the incidence of recurrence and metastasis of retiform Sertoli cell tumor with precision as we could not find any other case of Sertoli cell tumor of the testis with extensive retiform differentiation on record. In general, tumors with a retiform pattern are thought to have a worse prognosis than are tumors without a retiform component.  In our case, no evidence of recurrence or metastasis could be detected in the postoperative follow-up of 6 months.
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