This is a group of rare tumors of the testis derived from the interstitial cells. Sertoli cells, together with the Leydig cells comprise the majority of these cells. These tumors may present with hormonal manifestations. As as group, sex-cord stromal tumors represent approximately 4% of all testicular tumors.OUTLINE
Epidemiology Disease Associations Pathogenesis Gross Appearance and Clinical Variants Histopathological Features and Variants Laboratory/Radiologic/Other Diagnostic Testing Special Stains/
Differential Diagnosis Prognosis and Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Androblastoma AGE RANGE-MEDIAN Mean 45 years INCIDENCE
Stromal testis tumors in children: a report from the prepubertal testis tumor registry.
Thomas JC, Ross JH, Kay R.
Urological Institute, Section of Pediatric Urology, Cleveland Clinic Foundation, Cleveland, Ohio.
J Urol 2001 Dec;166(6):2338-40 Abstract quote
PURPOSE: Stromal testis tumors are rare and generally exhibit a benign behavior in prepubertal patients. We reviewed the Prepubertal Testis Tumor Registry to elucidate further the behavior of these tumors.
MATERIALS AND METHODS: Epidemiological and clinical information on stromal testis tumors was compiled and reviewed from the Prepubertal Testis Tumor Registry. In addition, original pathology reports were requested for all patients registered as having undifferentiated stromal tumors.
RESULTS: There were 43 patients registered with stromal tumors. Of the 21 patients with unspecified stromal tumors pathology reports were obtained on 11. Eight patients had truly mixed or undifferentiated stromal tumors. Mean patient age at presentation was 38 months (Leydig cell 70, Sertoli cell 52.5, juvenile granulosa cell 1.5 and mixed/undifferentiated 41.2). No patient with a Leydig cell, Sertoli cell or juvenile granulosa cell tumor had metastases at presentation or metastatic disease during an average 24.6 months of followup. One undifferentiated tumor demonstrated malignant behavior by presenting with metastatic disease. Pathological examination revealed a poorly differentiated tumor with extension into the adjacent tunica and frequent mitotic figures. While other stromal tumors displayed mitotic figures, none showed local invasion.
CONCLUSIONS: Stromal testis tumors are rare. Data from the Prepubertal Testis Tumor Registry confirms the benign behavior of most of these tumors. However, undifferentiated stromal tumors may exhibit metastatic behavior. A high index of suspicion is appropriate when there are a large number of mitotic figures, the tumor is poorly differentiated or when local invasion is present in the primary tumor. Metastatic evaluation and close followup are warranted for this select group of patients.
DISEASE ASSOCIATIONS CHARACTERIZATION PEUTZ-JEGHERS SYNDROME
Peutz-Jeghers syndrome with feminizing sertoli cell tumor.
Cantu JM, Rivera H, Ocampo-Campos R, Bedolla N, Cortes-Gallegos V, Gonzalez-Mendoza A, Diaz M, Hernandez A.
Cancer 1980 Jul 1;46(1):223-8 Abstract quote
A case involving a 6-year-old boy with Peutz-Jeghers syndrome and an unilateral feminizing Sertoli cell tumor is described. Endocrinologic studies revealed consistently high plasma and urine levels of estrogens and normal levels of testosterone and dihydrotestosterone. The increased levels of estrogens did not show changes that could be correlated with exogenous gonadotropin administration, thus indicating an autonomous nature.
The histopathologic studies of nontumorous testicular tissue revealed changes in the seminiferous tubules which suggested that estrogens, directly or indirectly, may have had both stimulating and atrophying effects.
It is concluded that gonadal tumors are in additional manifestation of the Peutz-Jeghers syndrome gene in both male and female patients.
Feminizing Sertoli cell tumors in boys with Peutz-Jeghers syndrome.
Young S, Gooneratne S, Straus FH 2nd, Zeller WP, Bulun SE, Rosenthal IM.
Department of Pathology, Cook County Hospital, Chicago, IL.
Am J Surg Pathol 1995 Jan;19(1):50-8 Abstract quote
We report the pathology findings in two cases of multicentric Sertoli cell testicular tumors in two young boys with probable Peutz-Jeghers syndrome.
Four cases of such tumors occurring in boys with Peutz-Jeghers syndrome were previously reported. Each of the two boys reported in this paper had prominent gynecomastia, rapid growth, and advanced bone age. Serum levels of estradiol were markedly elevated. Anti-mullerian hormone was measured in the serum of one of the boys and was in the normal range for age. Bilateral orchiectomy was performed in each case because the neoplastic growth would most likely result in sterility, and curtailment of height potential was threatened from continued elevation of estradiol levels.
Microscopically, greatly enlarged seminiferous tubules packed with ovoid Sertoli-like cells were present. Prominent eosinophilic basement membrane surrounded the tubules and intersected between the cells, forming hyalinized ovoid globules and microcalcifications.
Ultrastructure revealed lamination of basement membranes surrounding adjacent cells, ovoid cells with abundant cytoplasm, and limited smooth endoplasmic reticulum. Studies of testicular tumor tissue from both cases revealed increased transcription of the aromatase cytochrome P450 gene using promoter II, the promoter directing aromatase expression in the normal ovary and testis. The levels of transcripts were comparable to corpus luteum, thus resulting in increased estrogen synthesis. Transcripts specific for placental-type aromatase promoters (I.1 and I.2) were not detected in significant levels in these tumors.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Sertoli-Leydig cell tumors: a clinicopathologic study of 34 cases.
Roth LM, Anderson MC, Govan AD, Langley FA, Gowing NF, Woodcock AS.
Cancer 1981 Jul 1;48(1):187-97 Abstract quote
Thirty-four cases of Sertoli-Leydig cell tumor were studied.
All tumors were limited to the ovary at the time of initial surgery. Eight tumors were well differentiated, 15 were of intermediate differentiation, and 11 were poorly differentiated. Six cases contained heterologous elements. The less differentiated tumors occurred in patients with a lower median age and were more likely to produce androgenic manifestations. Follow-up of one year or longer was obtained in 15 patients, with an average follow-up in these patients of 6.1 years. Only one patient, who had a poorly differentiated tumor, died of the neoplasm in this series. Although follow-up was limited in this study, our findings suggest that the better differentiated tumors have a relatively favorable prognosis.
This neoplasm is composed of sex-cord and stromal elements, and its components have the capacity to a greater or lesser extent to recapitulate the cells of the testis at different stages of development.
Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases.
Young RH, Koelliker DD, Scully RE.
The James Homer Wright Pathology Laboratories of the Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.
Am J Surg Pathol 1998 Jun;22(6):709-21 Abstract quote
Sixty Sertoli cell tumors of the testis, excluding large cell calcifying and sclerosing subtypes, are described.
Patient age ranged from 15 to 80 years (mean, 45 years). The initial manifestation was usually a testicular mass; in 14 cases it had been enlarging slowly for a period of up to 14 years (mean 3.7 years). Only five patients had testicular pain. Four patients had metastatic disease at the time of presentation. All the tumors were unilateral and ranged from 0.3 cm to 15 cm (mean 3.6 cm). They were typically well circumscribed. Sectioning usually disclosed firm, tan-gray, white, or yellow tissue with areas of hemorrhage and a minor cystic component in approximately one third.
Microscopic evaluation usually revealed diffuse sheets or large, nodular aggregates of tumor cells, within which solid or hollow, sometimes dilated, tubules and, less often, cords were usually at least focally identifiable. A relatively acellular, often vascular, fibrous to hyalinized stroma was frequently conspicuous. The tumor cells typically had moderate amounts of pale to lightly eosinophilic cytoplasm, but 10 tumors had cells with abundant eosinophilic cytoplasm. Large cytoplasmic vacuoles were prominent in 26 tumors. Nuclear atypicality was absent or mild in 54 cases, moderate in 4 cases, and marked in 2 cases. Mitotic rate ranged from less than 1 to 21 per 10 high power fields, with 50 tumors having no or only rare mitoses. Vascular space invasion was present in 11 cases and was prominent in 8.
Follow-up of more than five years (average 8.4 years), or until evidence of metastasis was seen, was available for 16 patients. Nine were alive and well with no evidence of disease. Four were alive with disease and three died of disease.
The pathologic features that best correlated with a clinically malignant course were as follows: a tumor diameter of 5.0 cm or greater, necrosis, moderate to severe nuclear atypia, vascular invasion and a mitotic rate of more than 5 mitoses per 10 high power fields. Only one of nine benign tumors for which follow-up data of 5 years or more were available had more than one of these features, whereas five of seven malignant tumors had at least three.
Large cell calcifying Sertoli cell tumor
Large cell calcifying Sertoli cell tumor of the testis. A clinicopathologic, immunohistochemical, and ultrastructural study of two cases.
Tetu B, Ro JY, Ayala AG.
Department of Pathology, L'Hotel-Dieu de Quebec, Canada.
Am J Clin Pathol 1991 Dec;96(6):717-22 Abstract quote
The clinicopathologic, immunohistochemical, and electron microscopic study of two patients with large cell calcifying Sertoli cell tumors (LCCSCT) of the testis is reported to elucidate the histogenesis of this rare tumor.
Both tumors occurred in young individuals (16 and 32 years); case 1 was found incidentally, and the patient in case 2 presented with a 3-4-week history of testicular pain. Evidence of testosterone production was demonstrated in one case. In the same case, at the ultrastructural level Charcot-Bottcher crystalloids were observed.
These data support previous reports that LCCSCT shows Sertoli cell differentiation.
Large cell calcifying Sertoli cell tumour of the testis.
Plata C, Algaba F, Andujar M, Nistal M, Stocks P, Martinez JL, Nogales FF.
Department of Pathology, University Hospital, Granada, Spain.
Histopathology 1995 Mar;26(3):255-9 Abstract quote
Five cases of large cell calcifying Sertoli cell tumour of the testis not associated with complex dysplastic syndromes are reported.
The age of the patients ranged from 13 to 34 years and all the tumours were histologically similar, having large, isomorphic, non-mitotic, eosinophilic Sertoli cells with foci of calcification. Flow cytometry demonstrated the cells to be diploid or hypodiploid. All cases were positive for vimentin and focally positive for low molecular weight keratin.
The present cases, together with a review of the 22 previously reported tumours, demonstrate that there are two clear cut types of large cell calcifying Sertoli cell tumour; those which are associated with complex dysplastic syndromes and which are bilateral and multifocal, and those which are not associated and are unilateral and focal.
Prognosis in all of our cases was uniformly good despite invasion of the rete testis in two cases. It is considered that conservative resection of the tumour is the treatment of choice in cases not associated with complex dysplastic syndromes, since the malignancy rate is low.
Large cell calcifying Sertoli cell tumor of the testis: contrasting features of six malignant and six benign tumors and a review of the literature.
Kratzer SS, Ulbright TM, Talerman A, Srigley JR, Roth LM, Wahle GR, Moussa M, Stephens JK, Millos A, Young RH.
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, USA.
Am J Surg Pathol 1997 Nov;21(11):1271-80 Abstract quote
We report six malignant and six benign large cell calcifying Sertoli cell tumors of the testis and compare the features of malignant and benign cases based on these cases and those in the literature.
All the tumors in this report consisted of sheets, nests, solid tubules, and cords of eosinophilic cells, with focal calcifications, as well as a substantial neutrophilic infiltrate in 11 of them.
Analysis of our cases and those in the literature showed that the malignant tumors were unilateral and solitary and occurred at a mean age of 39 years (range 28-51 years), whereas the benign neoplasms were bilateral and multifocal in 28% of cases and occurred at a mean age of 17 years (range 2-38 years). Only one malignant tumor occurred in a patient with evidence of a genetic syndrome (Carney syndrome), whereas 36% of benign tumors had various genetic syndromes or endocrine abnormalities. Most of the tumors in the latter cases were bilateral and multifocal.
There were strong associations of malignant behavior with size >4 cm, extratesticular growth, gross or microscopic necrosis, high-grade cytologic atypia, vascular space invasion, and mitotic rate greater than three mitoses per 10 high-power fields.
All malignant cases exhibited at least two of these features, whereas all benign cases lacked any of them. The presence of any one of these features in a solitary large cell calcifying Sertoli cell tumor, especially in a patient >25 years of age, should be viewed as suspicious for malignant behavior, whereas the presence of two or more of these features indicates a strong probability of a malignant course.
"Low" percentages (< or =35%) of tumor cells staining for proliferating cell nuclear antigen (PCNA) also may correlate with benign behavior, but some benign tumors have high PCNA values. Ki-67 values (MIB-1 antibody) did not correlate with biologic behavior, nor did immunostains for p53 protein.
Large cell calcifying Sertoli cell tumor of the testis: a clinicopathological, immunohistochemical, and ultrastructural study of two cases.
Cano-Valdez AM, Chanona-Vilchis J, Dominguez-Malagon H.
Department of Surgical Pathology, Instituto Nacional de Cancerologia, Mexico, DF.
Ultrastruct Pathol 1999 Jul-Aug;23(4):259-65 Abstract quote
Large cell calcifying Sertoli cell tumor of the testis (LCCSCT) is a rare tumor that is usually benign and multifocal. It may be associated with hereditary endocrine anomalies such as Carney's and Peutz-Jeghers syndromes. Malignant forms are exceptional.
Two cases of LCCSCT, one malignant and one benign are described. Both were composed of cords and trabeculae of large polygonal cells embedded in a myxoid and fibrous stroma with areas of calcification. The malignant tumor showed nuclear atypia, necrosis, and abundant mitoses. The cells were positive for vimentin and S-100 protein, and the intercellular space for laminin and collagen type IV.
By electron microscopy, nucleolonemas and multilayered basal lamina were seen. The benign tumor was positive for vimentin and S-100 protein, and ultrastructurally showed less basal lamina.
Malignant large cell calcifying Sertoli cell tumor of the testis (LCCSCTT). Report of a case in an elderly man and review of the literature.
Bufo P, Pennella A, Serio G, Mastropasqua MG, Cenacchi G, Rogatsch H, Mikuz G.
Institute of Pathological Anatomy, University of Bari, Italy.
Pathologica 1999 Apr;91(2):107-14 Abstract quote
Malignant Large Cell Calcifying Sertoli Cell Tumor of the Testis (LCCSCTT) is a rare histological variant of sex cordstromal tumors. It usually arises in young males, sometimes is associated with endocrine abnormalities and has a benign course. It is exceptional in elderly men and the outcome is rarely fatal.
We report a case of LCCSCTT in a 73 year-old man with fatal outcome. The tumor involved the right testis and several areas of the tunica albuginea were grossly invaded. Serum levels of HCG, LH and testosterone were normal. Lymphoangiography performed after orchiectomy showed an involvement of the iliac and preaortic lymph nodes. X-ray of the chest showed no lung metastases. A thorough study of the light microscopic, immunohistochemical and ultrastructural appearances was performed. Immunohistochemistry revealed positivity to vimentin, S-100 and NSE.
Our observations confirm the previous findings concerning malignant LCCSCTT and point out the histogenesis of the tumor from Sertoli cells.
VARIANTS INTRATUBULAR LARGE CELL HYALINIZING SERTOLI CELL NEOPLASIA
- Intratubular Large Cell Hyalinizing Sertoli Cell Neoplasia of the Testis: A Report of 8 Cases of a Distinctive Lesion of the Peutz-Jeghers Syndrome.
*Department of Pathology, Indiana University School of Medicine, Indianapolis, IN †Department of Pathology, Cedars Sinai Health System, Los Angeles, CA ‡The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
- Am J Surg Pathol. 2007 Jun;31(6):827-835. Abstract quote
We report the clinical and pathologic features of 8 boys with Peutz-Jeghers syndrome who had distinctive testicular lesions. The patients were 4 to 13 years of age (mean, 6.5 y), and all had gynecomastia, which was the presenting feature in 7. Physical examination demonstrated bilateral testicular enlargement in the absence of a discrete mass. Advanced bone age and elevated serum estradiol were demonstrated in 3 and 4 cases, respectively.
Testicular biopsy, performed in all cases, usually showed no gross abnormality, but on microscopic examination there were patchily distributed clusters of expanded seminiferous tubules that contained large Sertoli cells with vacuolated to eosinophilic cytoplasm admixed with globular deposits of basement membrane that extended from a thickened peritubular basement membrane. Small, focal calcifications occurred in 3 cases; no invasive tumor was present in any of the cases. Follow-up was available in 5 patients after biopsy, and none showed evidence of progression at 10 months to 5 years (median, 4 y). Review of the previously reported cases of testicular lesions in Peutz-Jeghers patients verified a low frequency of invasive tumors (27%) and no known case with metastasis.
The testicular lesions seen in patients with Peutz-Jeghers syndrome mostly represent multifocal intratubular neoplasia of large Sertoli cells with unique morphology distinct from other lesions such as the large cell calcifying Sertoli cell tumor and sex cord tumor with annular tubules. The process usually remains confined to the tubules for prolonged intervals (years), but it may occasionally progress to invasive large cell Sertoli cell tumors with or without associated calcification.
This indolent course justifies management by careful follow-up, including ultrasound examination, rather than orchiectomy in the majority of cases. Orchiectomy is indicated when there is evidence of an invasive tumor and may be necessary to control hormonal manifestations.
Sertoli Cell Proliferations of the Infantile Testis An Intratubular Form of Sertoli Cell Tumor?
Marcela Venara, M.D.; Rodolfo Rey, M.D., Ph.D.; Ignacio Bergadá, M.D.; Hernán Mendilaharzu, M.D.; Stella Campo, M.Sc.; Héctor Chemes, M.D., Ph.D.
From the Laboratory of Testicular Physiology and Pathology, Endocrinology Division (M.V., R.R., I.B., S.C., H.C.), Children's Hospital “Ricardo Gutiérrez,” CONICET. Buenos Aires, Argentina; and the Department of Endocrinology (H.M.), Pediatric Hospital “J.P. Garrahan,” Buenos Aires, Argentina.
Am J Surg Pathol 2001;25:1237-1244 Abstract quote
We report on six boys with intratubular Sertoli cell proliferations (ISCPs), studied by routine histologic methods, electron microscopy, and immunohistochemistry of anti-müllerian hormone (AMH), inhibin -subunit, 3-hydroxysteroid dehydrogenase (3-HSD), proliferative cellular nuclear antigen, and p53, and carefully followed for extended periods with periodic clinical examinations, testicular ultrasonographies, and determinations of serum levels of AMH and inhibin B.
Peutz–Jeghers syndrome was found in four of six patients, and gynecomastia occurred in five of six patients. One boy had isosexual pseudoprecocity. ISCPs were observed as multiple foci of seminiferous tubules with large and proliferated Sertoli cells replacing germ cells and limited by the basement membrane. Mitotic figures, atypia, and/or interstitial invasion were not observed. Bilateral ISCPs were the only pathologic finding in three patients (patient nos. 1–3) and were associated with a microscopic tumor that resembled a large-cell calcifying Sertoli cell tumor (LCCSCT) in a fourth patient (patient no. 4). In the two remaining patients (patient nos. 5 and 6) ISCPs and LCCSCT were found in both testes. Ultrastructural examination showed large Sertoli cells, with round nuclei, sparse organelles, and some glycogen. Inhibin -subunit immunolocalization was positive in the five patients in whom it was determined (patient nos. 2–6), AMH was positive in those ISCPs associated with tumors (patient nos. 4–6) and negative in isolated ISCPs (patient nos. 2 and 3); 3-HSD and PCNA were variable, and p53 was negative in all ISCPs. Patient nos. 1–4 have been followed for 2–19 years. One of them is currently entering puberty, the other two have already completed puberty and have testes of normal size, and the remaining one is an adult with clinically normal testes and sperm production. None of these patients had evidence of tumor development during follow-up as shown by serial ultrasonographies and serum levels of AMH and inhibin B. Patient nos. 5 and 6 who had bilateral ISCPs and LCCSCT were orchidectomized and evolved for 2–10 years after surgery without tumor recurrence.
The prognostic significance of ISCPs, particularly when they are the only pathologic finding in a testicular biopsy, is a matter of controversy. Based on the long normal evolution, we recommend a conservative approach to therapy. The bilateral and multicentric character of ISCPs and their association with Sertoli tumors and Peutz–Jeghers syndrome suggest that they represent either proliferative lesions with tumorigenic potential or the intraepithelial stage in the evolution of some testicular Sertoli cell tumors.
Malignant sertoli cell tumors of the testis: a study of 13 examples of a neoplasm frequently misinterpreted as seminoma.
Henley JD, Young RH, Ulbright TM.
Departments of Pathology (J.D.H., T.M.U.), Indiana University School of Medicine, Indianapolis, Indiana, and the James Homer Wright Pathology Laboratories (R.H.Y.), Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, U.S.A.
Am J Surg Pathol 2002 May;26(5):541-50 Abstract quote
The distinction of Sertoli cell tumors from seminoma is critical to ensure proper treatment. Although usually straightforward, we highlight herein 13 malignant Sertoli cell tumors of the testis with light microscopic features that mimicked seminoma.
All of the cases were received in consultation, 10 with submitting diagnoses of seminoma, usually of classic type, but three cases of spermatocytic type. Patients ranged from 15 to 80 years of age (median 37 years); all presented with testicular masses. The tumors were typically firm, white to yellow-tan, and often had foci of hemorrhage. The dominant microscopic pattern was nested or sheet-like, with some tumors having secondary patterns of trabeculae-solid tubules, hollow tubules, and pseudofollicles.
Tumor cells were polygonal with conspicuous clear cytoplasm in 12 cases; the cytoplasm was focally eosinophilic in 10 cases, but this was never conspicuous. Nine tumors had cytoplasmic vacuoles, and three of four that were investigated stained for intracytoplasmic glycogen. Nuclei were small (5) to medium-sized (8), round-to-oval (13), and vesicular with irregular contours (11). Nucleoli were present in 11 tumors (six small; five large). Stromal fibrosis (12) and lymphoid infiltrates (10) were conspicuous, and tumor necrosis (11) and vascular invasion (8) also were seen. Mitotic figures ranged from <1 to 21/10 high power fields (HPF) (median 1/10 HPF). Staining for inhibin-alpha, epithelial membrane antigen, and cytokeratin (AE1/AE3) was positive in four of four, six of six, and three of six cases, respectively; placental alkaline phosphatase was negative in all five tumors investigated.
The nested growth pattern, prominence of clear cells, lymphoid infiltrate, inconspicuous tubular differentiation, cytoplasmic glycogen, and prominent nucleoli caused these tumors to be mistaken for seminomas. The smaller, less pleomorphic nuclei of Sertoli cell tumors, their lower mitotic rate, and the absence of intratubular germ cell neoplasia are helpful differential features.
Immunohistochemistry is a useful adjunct in confirming the diagnosis of Sertoli cell tumor, but only if the overlapping features are appreciated by conventional microscopy and the diagnosis of Sertoli cell tumor included in the differential.
Testicular Sertoli cell tumor with a heterologous sarcomatous component: immunohistochemical assessment of Sertoli cell differentiation.
Gilcrease MZ, Delgado R, Albores-Saavedra J.
Department of Pathology, University of Texas Southwestern Medical Center, Dallas 75235-9072, USA.
Arch Pathol Lab Med 1998 Oct;122(10):907-11 Abstract quote
OBJECTIVE: Immunohistochemical staining is reported to be useful in distinguishing ovarian Sertoli-stromal cell tumors from carcinosarcomas. To assess Sertoli cell differentiation in a rare malignant biphasic testicular tumor, we compared the immunophenotypic profile of the tumor with that of Sertoli cell nodules and adenomas and mullerian carcinosarcomas.
DESIGN: Immunohistochemical staining was performed on 6 testes (4 with hyperplastic Sertoli cell nodules, 2 with Sertoli cell adenomas) and 7 carcinosarcomas (6 involving the uterus, 1 involving the uterus and ovary) using primary monoclonal antibodies AE1/AE3, CAM 5.2, CA 19.9, and antibodies directed against epithelial membrane antigen, carcinoembryonic antigen (monoclonal and polyclonal), S100, placental alkaline phosphatase, and inhibin. These staining results were compared with those of the index case.
RESULTS: All testes showed positive staining for inhibin and vimentin in the Sertoli cells of the nodules and adenomas. One Sertoli cell nodule showed focal staining with AE1/AE3 and CAM 5.2. Both adenomas showed focal positive staining for S100. All nodules and adenomas were negative for epithelial membrane antigen, monoclonal and polyclonal carcinoembryonic antigen, CA 19.9, and placental alkaline phosphatase. In contrast, the carcinomatous areas of the carcinosarcomas were all negative for inhibin but exhibited positive staining for AE1/AE3, CAM 5.2, and epithelial membrane antigen. The carcinosarcomas showed variable expression of vimentin, S100, carcinoembryonic antigen, CA 19.9, and placental alkaline phosphatase. The epithelial component of the tumor from the index case showed strong diffuse staining for inhibin and vimentin and only very faint focal staining with AE1/AE3 and CAM 5.2. The epithelial component was negative for epithelial membrane antigen, monoclonal and polyclonal carcinoembryonic antigen, S100, CA 19.9, and placental alkaline phosphatase.
CONCLUSIONS: The immunohistochemical findings in the index case support the diagnosis of Sertoli cell tumor with a heterologous sarcomatous component over carcinosarcoma. Inhibin seems to be the best single marker for Sertoli cell differentiation. To our knowledge, only 1 other case of this rare testicular tumor has been reported in the literature.
Sclerosing Sertoli cell tumor of the testis: a distinct histological subtype.
Department of Urology, Marshfield Clinic, Wisconsin, USA.
J Urol 1995 Nov;154(5):1756-8 Abstract quote
PURPOSE: A case of sclerosing Sertoli cell tumor of the testis is reported. The histological and clinical features are compared to those of other Sertoli cell tumor subtypes.
MATERIALS AND METHODS: The current urological and pathological literature was reviewed.
RESULTS: The tumor was identified incidentally in a 35-year-old white man. Pathological examination was performed and the patient was well at 9 months.
CONCLUSIONS: Sertoli cell tumors are heterogeneous and not accurately considered a uniform entity. The currently recognized variants differ in apparent malignant potential as well as the association with disease processes in other unrelated organ systems.
Utility of immunostaining for S-100 protein subunits in gonadal sex cord-stromal tumors, with emphasis on the large-cell calcifying Sertoli cell tumor of the testis.
Tanaka Y, Carney JA, Ijiri R, Kato K, Miyake T, Nakatani Y, Misugi K.
Division of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.
Hum Pathol 2002 Mar;33(3):285-9 Abstract quote
This study concerns the immunohistochemical localization of S-100 alpha, S-100 beta, and whole brain S-100 (wbS-100) in testicular large-cell calcifying Sertoli cell tumor (LCCSCT).
We examined 8 LCCSCTs (7 benign and 1 malignant), 6 Sertoli cell tumors not otherwise specified (SCTs-NOS), 6 Leydig cell tumors (LCTs), 5 ovarian Sertoli-Leydig cell tumors (SLCTs), and 7 gonadoblastomas (GBLs). The 8 LCCSCTs showed immunoreactivity for S-100 alpha, S-100 beta, and wbS-100. Five of the 6 LCTs and the Leydig cell components in the ovarian SLCTs stained positively for S-100 alpha and wbS-100 but were negative for S-100 beta. SCTs-NOS and the Sertoli cell components in the SLCTs occasionally showed focal and weak/moderate positivity for S-100 alpha, S-100 beta, and wbS-100. Sex cord cells of the GBLs were positive for S-100 beta and wbS-100 and negative for S-100 alpha. Germ cell elements of the GBLs were negative for S-100 alpha, S-100 beta, and wbS-100. In nonneoplastic testicular parenchyma adjacent to the above-mentioned tumors, there was S-100 alpha reactivity in Leydig cells, rete testis, and a few Sertoli cells. S-100 beta reactivity was seen in a few Sertoli cells, Schwann cells, and some endothelial cells. WbS-100 reactivity was present in Leydig cells, a few Sertoli cells, rete testis, Schwann cells, and some endothelial cells.
The results indicate that S-100 alpha and S-100 beta can potentially be used as immunohistochemical markers for LCCSCT, especially when differentiating it from LCT, which may mimic LCCSCT on routine histopathology. Although the biological significance of both S-100 subunits expression in LCCSCT remains unknown, these notable calcium-binding proteins may be associated with the characteristic calcification in LCCSCT through regulation of calcium levels in the tumor cells.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
Sertoli cell tumor in a prepubertal boy mimicking testicular torsion.
Tan M, Uygur C, Ozer E, Erol D.
Clinic of Urology, Ministry of Health Ankara Hospital, Ankara, Turkey.
Urol Int 2000;65(4):211-3 Abstract quote
A 9-year-old boy presented with left, intermittent testicular pain that was present for 3 days. On physical examination, left testis was grossly enlarged and firm but mildly tender. Serum alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal range. Color doppler ultrasonography which was performed to rule out testicular torsion revealed an intratesticular mass located at the upper pole of left testis and left radical orchiectomy was performed. The histopathological diagnosis was Sertoli cell tumor.
Sex cord-stromal tumors of the testis with entrapped germ cells: a lesion mimicking unclassified mixed germ cell sex cord-stromal tumors.
Ulbright TM, Srigley JR, Reuter VE, Wojno K, Roth LM, Young RH.
Department of Pathology & Laboratory Medicine, Indiana University Medical Center, Indianapolis 46202-5280, USA.
Am J Surg Pathol 2000 Apr;24(4):535-42 Abstract quote
The authors describe 10 sex cord-stromal tumors of the testis that incorporated germ cells, thereby mimicking the unclassified type of mixed germ cell sex cord-stromal tumor (MGCSCST).
These neoplasms occurred in patients from 3 to 48 years old (mean age, 26 years) who presented with testicular masses.
On microscopic examination, nine tumors had a combination of tubular and cord-like arrangements of sex cord cells with transition to spindle-shaped tumor cells. They were diagnosed as either unclassified sex cord-stromal tumors (n = 5) or Sertoli-stromal cell tumors (n = 4). One tumor was a pure Sertoli cell tumor. The admixed germ cells were usually at the periphery and in clusters, but occasionally were in the center or more diffuse. In nine patients the germ cells resembled spermatogonia, having round nuclei with uniform, dusty chromatin and inconspicuous or small nucleoli. None of these cells stained with a variety of markers used for neoplastic germ cells, and in one case in which the non-neoplastic Sertoli cells were strongly reactive for inhibin but the neoplastic Sertoli cells were not, all the germ cells within the tumor occurred adjacent to inhibin-positive Sertoli cells.
With static cytophotometry, a diploid deoxyribonucleic acid content was found in these germ cells in the two investigated cases. In one case the germ cells had the morphologic appearance of seminoma cells and they stained positively for the markers of neoplastic germ cells. This case was interpreted as a "collision" tumor between a Sertoli cell tumor and a seminoma.
The authors conclude that sex cord-stromal tumors with entrapped germ cells of the testis are more common than unclassified MGCSCSTs--a bona fide testicular example of which has not been seen by any of the authors.
Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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