Characteristic ultrasound appearance of a rare testis tumor: Bilateral multiple large-cell calcifying Sertoli cell tumor.

Characteristic ultrasound features of large cell calcifying Sertoli cell tumor (LCCSCT), including hypoechoic masses with amorphous coarse calcifications can aid in differentiating this tumor from other entities. Bilateral multiple LCCSCTs almost always show a benign course; therefore, defining the diagnosis with sonographic findings is crucial to avoid unnecessary orchiectomy.

Possible hints and pitfalls in diagnosing Peutz-Jeghers syndrome.

Background Peutz-Jeghers syndrome (PJS) is characterized by gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition. Patients with PJS can develop large calcifying Sertoli cell tumors (LCSTs). Case presentation A patient presented at 3 years of age with delayed development, hypermobility and later also with tall stature and advanced bone age. Extensive endocrine evaluation, mutation analysis of genes associated with connective tissue disorders and a single nucleotide polymorphism (SNP) array showed no abnormalities. At 8 years of age, gynecomastia developed as well as pigmentations on the lips, both of which are associated with PJS. Mutation analysis showed a heterozygous deletion of the whole STK11 gene confirming PJS. Testicular ultrasound confirmed the presence of LCSTs. Interestingly, the previously performed SNP array did not report deletion of the STK11 gene. Conclusions We advise excluding LCSTs in children with tall stature and advanced bone age where more common causes have been eliminated. Although STK11 deletions are documented in control databases, reporting the deletion of this gene even in the absence of a phenotype is advised for patient management.

Organ-sparing surgery for large cell calcifying Sertoli cell tumour in a patient with Carney complex.

Carney complex is a rare genetic disease characterised by a complex of myxomas, spotty pigmentation and endocrine overactivity. At diagnosis, about one-third of male patients presents with testicular tumours, namely large cell calcifying Sertoli cell tumours, which are often multicentric and/or bilateral and have a low malignant potential. Although radical orchiectomy is the gold standard for the treatment of testicular neoplasms, a conservative approach with partial orchiectomy or tumourectomy may be the best treatment option for these patients, allowing the preservation of endocrine function, fertility and body image. We present a case of a 19-year-old man with a known history of Carney complex with early identification of a small testicular tumour treated with organ-sparing surgery.

[Testicular nodules of infertile men and contrast enhanced ultrasonography: preliminary study]. / Nodules testiculaires de l'homme infertile et échographie de contraste: étude préliminaire.

INTRODUCTION: The incidence of testicular nodules discovered during infertility evaluation is increasing. These nodules are suspicious of malignancy. There is no paraclinical examination which allows histological orientation to these nodules. The recommendations propose priority treatment by total orchidectomy. PATIENTS AND METHODS: Through a retrospective cohort study of infertile patients, our goal is to study the enhancement of testicular nodules after injection of ultrasound contrast. The secondary objective is to determine whether CEUS may argue in favor of conservative treatment. From june 2010 to march 2013, 24 patients had underwent ultrasound contrast study of abnormal testicular parenchyma detected prior to infertility evaluation carried ultrasound. The characteristics of ultrasound enhancement were correlated with the pathological findings of surgical patients and proposed treatments (surgery or surveillance). RESULTS: Fifteen patients were followed up, 9 were operated (7 partial orchidectomies, 2 total orchidectomies). Histological analysis found four Leydig cell tumors, 2 Sertoli cell tumors and 3 seminomas. No adverse changes were noted during the follow-up. This study showed a typical semiology of early, intense and homogeneous enhancement with a phenomenon of wash in 100% of Leydig cell tumors. All Leydig cell tumors have been treated by partial orchidectomy. Seminomas have intense enhancement in 100% of cases. There was a phenomenon of wash in 2 out of 3 cases. When a wash in was described, it was always described as heterogeneous. All seminomas were finally treated by total orchidectomy. The sensitivity and positive predictive value of ultrasound intense enhancement for the diagnosis of testicular cancer was 89% (Se) and 80% (PPV). CONCLUSION: There is a semiology of ultrasound enhancement of testicular nodules with features that can guide in favor of a malignant tumor, seminoma or Leydig cell tumor. If a prospective study was undertaken, these arrangements could guide us to treatments promoting preservation of the testicular parenchyma.

[Large cell calcifying Sertoli cell tumour: A case report]. / La tumeur à cellules de Sertoli à grandes cellules calcifiantes : à propos d'un cas.

A 19-year-old male Caucasian, without prior medical history, noticed a painless right testicular mass. Physical examination revealed neither gynecomastia nor abnormal skin pigmentation. Serum alpha-fetoprotein, ß-HCG and testosterone levels were normal. Sonography depicted an intratesticular diffusely hyperechoic lesion with acoustic shadowing. The patient underwent right orchiectomy. Histology revealed a benign large cell calcifying Sertoli cell tumour. This tumour is rare and may be associated with genetic abnormalities.

Sertoliform cystadenoma: a rare benign tumour of the rete testis.

Sertoliform cystadenoma of the rete testis represents an uncommon benign tumour. They appear in patients from 26 to 62 years of age. We describe a case of a 66-year-old man with a tumour in the area of the epididymal head. The tumour markers were not increased. Under the assumption of a malignant testicular tumour an inguinal orchiectomy was performed. The cut surface of this tumour was of grey/white color and showed small cysts. The tumour consisted of two compartments. The epithelial like tumour cells showed a sertoliform growth pattern and cystic dilatations. In between the tumour cells repeatedly actin expressing sclerotic areas could be recognized as the second tumour component. Proliferative activity was not increased. Immunohistochemically the tumour cells were positiv for inhibin, S-100, and CD 99. Alpha feto protein (AFP), human chorionic gonadotropin (ß-HCG) and placental alkaline phosphatase (PLAP) as well as synaptophysin, epithelial membrane antigene (EMA), and BCL-2 were not expressed. As far as we know this is the sixth reported case of this tumour. Because of the benign nature of this tumour the correct diagnosis is important for the intra- and postoperative management. Here we present a case of this rare tumour and discuss potential differential diagnosis. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1956026143857335.

An unexpected abdominal tumour in an elderly woman.

We report the case of a 73-year-old woman who presented with pain and an abdominal mass and was found to have an abdominal tumour of unexpected origin.

Sertoli-stromal cell tumor of the right ovary: radiological-pathological correlation.

A case of Sertoli-stromal cell tumor of the right ovary is reported. The patient was a 50-year-old perimenopausal woman with abdominal distention due to a large pelvic tumor. She had no signs of androgen excess. A large solid sponge-like tumor with multicystic areas throughout, in which there were some small hemorrhagic spots, was shown on magnetic resonance (MR) imaging. No endometrial thickening of the uterus was seen. Pathology examination revealed a Sertoli-stromal cell tumor with intermediate-to-poor differentiation. The edematous, watery, sponge-like appearance on the MR images correlated with the pathological findings.

[A case of Sertoli cell tumor].

We report a case of Sertoli cell tumor. A 33-year-old man visited our clinic with a complaint of painless left scrotal swelling on September 29th, 2003. An elastic firm induration larger than a hen's egg in size was palpable on the surface of the left testis. Tumor markers for testicular tumor such as human chorionic gonadotropin-beta, alpha fetoprotein, and lactate dehydrogenase were not elevated. However, ultrasound showed a low echoic mass in the left testis. Therefore, we performed left high orchiectomy under the diagnosis of left testicular tumor. Its histology showed Sertoli cell tumor. Neither recurrence nor metastasis has been detected for about 8 months after the operation.

Bilateral sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome.

Thirty-year-old woman with lower abdominal pain was operated due to adnexial mass. Cystectomy on right gonad revealed sertoli cell adenoma and simple serous cyst and left gonadal biopsy showed immature testis tissue. Later, laparoscopic left gonadectomy was made. Histopathology of the left gonad was consistent with sertoli cell adenoma.

Stromal testis tumors in infants. a report of two cases.

Gonadal stroma tumors account for 8% of pediatric testicular tumors and are therefore exceedingly rare. They generally exhibit a benign behavior. We report two consecutive cases of gonadal stroma tumors in infants. A 5-month-old boy presented with a Sertoli cell tumor and a 2-month-old boy with a juvenile granulosa cell tumor without systemic disease. Both were diagnosed incidentally during routine examinations. Organ-sparing surgery and radical orchiectomy, respectively, was the therapy of choice. Although neonatal testicular tumors are rare, they should be considered in the differential diagnosis of a newborn with a scrotal mass.

PET imaging of Sertoli cell tumor in androgen insensitivity syndrome.

The author presents a case of Sertoli cell tumor demonstrated incidentally by F-18 fluorodeoxyglucose positron emission tomography during imaging workup for suspected right-lung malignant neoplasm. This rare sex cord stromal tumor arose from the right gonad of a patient with a history of androgen insufficiency syndrome, also known as testicular feminization syndrome. The relationship of this phenotypic sexual disorder with Sertoli cell tumor is briefly reviewed and discussed.

Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?

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 alpha-subunit, 3beta-hydroxysteroid dehydrogenase (3beta-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 alpha-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); 3beta-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.

[A case of Sertoli cell tumor of the testis].

A 24-year-old man was admitted to our hospital with the complaint of a painless mass in the left testis. Gynecomastia was not present. The serum levels of alpha fetoprotein and human chorionic gonadotropin-beta were not elevated. Ultrasound sonography showed a hypoechoic lesion in the left testis. There was no evidence of retroperitoneal lymph node enlargement or distant metastasis on computerized tomography. With a diagnosis of left testicular tumor, left high orchiectomy was done. The tumor measured 10 x 11 mm in size within the testis and was histologically diagnosed as benign Sertoli cell tumor. Immunohistochemcal analysis revealed negative findings with the tumor markers inhibin, CAM 5.2, pancytokeratin, EMA, and PLAP. No adjuvant therapy was performed. Twenty-six cases of Sertoli cell tumor of the testis in the Japanese literature are reviewed.

Sertoli cell tumor in a prepubertal boy mimicking testicular torsion.

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.