Experience with testis sparing surgery for testicular teratoma

PURPOSE: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery. MATERIALS AND METHODS: We reviewed the pathology records at our institution for all testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We specifically examined the prepubertal incidence of teratoma, including epidermoid cysts, and our experience with testis preserving surgery. Preoperative and postoperative ultrasonography images were used to calculate the atrophy index following surgery. Patients were contacted for long-term followup. RESULTS: Of 77 primary testicular and paratesticular tumors 38 were diagnosed in prepubertal boys (age younger than 13 years) including 11 mature teratomas and 5 epidermoid cysts. Mean patient age at treatment was 34.4 months (range 4 months to 10 years). All boys presented with a painless scrotal mass, cystic foci within an intratesticular mass on ultrasound and a normal alpha-fetoprotein level. Of the 16 boys with benign teratomas 13 (81%) were treated with a testis sparing procedure. At a mean 7-year followup no patient has presented with recurrent tumor in the ipsilateral or contralateral testicle. Postoperative physical examination and scrotal ultrasound were obtained in 9 patients at a median followup of 10.2 months, and there was no evidence of testicular atrophy or persistent discomfort. CONCLUSIONS: Unlike previously published series based on tumor registries, benign teratoma was the most common pediatric testicular tumor treated at our institution. Our single institution experience with testis preservation and long-term followup confirms the role and safety of this technique. Testis sparing surgery remains our technique of choice for testicular teratoma.     

ORGAN SPARING SURGERY FOR MALIGNANT GERM CELL TUMOR OF THE TESTIS

PURPOSE: We evaluate the indication, technique and long-term outcome of organ preserving tumor resection rather than standard orchiectomy in 73 patients with bilateral testicular germ cell tumors or tumors of a solitary testicle. MATERIALS AND METHODS: Tumor resection was performed in 73 patients with 52 and 17 metachronous and synchronous bilateral testicular germ cell tumors, respectively, and 4 testicular germ cell tumors of a solitary testicle. Histology of the enucleated germ cell tumor revealed seminoma in 42 (57.5%) patients, embryonal carcinoma in 14 (19.2%), mature teratoma in 11 (15.1%), and mixed and combined germ cell tumors in 6 (8.2%). Mean tumor diameter was 15 mm. (range 5 to 30). Associated testicular intraepithelial neoplasia was diagnosed in 82% of patients who underwent local radiation with 18 Gy. RESULTS: After a median followup of 91 months (range 3 to 191) 72 (98.6%) patients had no evidence of disease and 1 died of systemic tumor progression. No local relapse occurred in 46 patients with associated testicular intraepithelial neoplasia treated with local radiation. However, local recurrence did develop in 4 patients after 3, 6, 12 and 165 months without radiation but all survived after undergoing inguinal orchiectomy. Testosterone levels were normal in 62 (84.9%) patients, hypogonadism developed in 7 (9.6%) and preoperative levels remained low in 4 (5.5%). Of the 10 patients who postponed local radiation for paternity reasons 5 had successfully fathered a child after organ sparing surgery. CONCLUSIONS: After a long-term followup of greater than 7 years organ sparing surgery represents a viable therapeutic approach to bilateral testicular germ cell tumor with an excellent postoperative outcome. Tumor enucleation might be considered a standard approach if the guidelines are respected, including cold ischemia, organ confined tumor less than 20 mm., multiple biopsies of the tumor bed, adjuvant local irradiation postoperatively to avoid local recurrence, close followup and high compliance.     

Testicular-sparing surgery for the prepubertal testicular tumor. Experience of two cases with large cell calcifying Sertoli cell tumors.

PURPOSE: We review prepubertal germ cell tumors of testis in our institute and the Japanese registry and present 2 cases with a large cell calcifying Sertoli cell tumor (LCCSCT) and discuss the possibility of testis-sparing surgery. MATERIALS AND METHODS: Incidence, age, pathology and clinical stages of prepubertal germ cell tumors are surveyed for 30 years at our department and 10 years of the malignant tumor registry of the Japanese Society of Pediatric Surgery. Two representative prepubertal boys with LCCSCT are presented. One of them was treated by partial orchiectomy. RESULTS: The majority of testicular germ cell tumors in the prepubertal age were composed of embryonal cell carcinoma/yolk sac tumors or teratoma, occurred in preschool age, were limited to clinical stage I and did not metastasize irrespective of histology. Benign behavior which included recovery from hormonal derangement, no tumor recurrence and negative antisperm antigen was observed in 2 cases with LCCSCT who underwent either radical orchiectomy or partial orchiectomy. CONCLUSION: Partial orchiectomy should be considered as a standard option in prepubertal schoolboys with a testicular mass if surgically feasible. This surgical treatment is safe and preserves fertility and is psychologically advantageous. It is not recommended for yolk sac tumors that may recur, however they are rare in prepubertal boys and can be differentiated preoperatively by prudent evaluation.     

Enucleation for prepubertal leydig cell tumor

PURPOSE: Leydig cell tumors in children are rare, comprising only 4% to 9% of all primary testis tumors in prepubertal males. Almost all of these boys present with isosexual precocious pseudopuberty associated with increased testosterone, low gonadotropin levels and a testis mass. We present our experience with testis sparing enucleation of Leydig cell tumor in prepubertal boys. MATERIALS AND METHODS: Two patients presented with isosexual precocious puberty at ages 6 and 9 years. Each patient had a well circumscribed, painless testicular mass, increased serum testosterone (101 and 444 ng/dl [normal 0 to 25]), normal gonadotropins and negative alpha-fetoprotein levels. Both patients underwent successful enucleation of the testis mass following proper testis oncological surgical principles. RESULTS: Both patients had normalization of the serum testosterone following enucleation of the Leydig cell tumor. At 9 and 44 months of followup they have maintained normal ipsilateral testicular volume compared to the contralateral gonad, and 1 patient entered puberty spontaneously at 1 year postoperatively. Neither patient suffered any morbidity, and both have presumably benefited from preservation of the involved gonad with preserved testicular volume. CONCLUSIONS: Prepubertal boys with isosexual precocious pseudopuberty, an isolated testis mass, increased testosterone and low or normal gonadotropin levels can reliably be diagnosed with Leydig cell tumors. Based on the ability to establish the diagnosis preoperatively and the universal benign behavior of unilateral, prepubertal Leydig cell tumor, we believe these patients are best treated with testis sparing enucleation of the tumor. In view of the high likelihood that this tumor in prepubertal boys is benign, a transscrotal surgical approach should be considered.     

Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis

PURPOSE: Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis. MATERIALS AND METHODS: We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years. We specifically reviewed our experience with JGCT in terms of presentation, surgical treatment and long-term outcome. We also reviewed the microscopic findings and histochemical techniques used to confirm the diagnosis. RESULTS: We identified 77 tumors during the defined interval, of which 3 (3.9%) were JGCTs. All 3 patients with JGCT were first noted to have a testis mass soon after birth. All presented with a firm, unilateral testicular mass. Ultrasonographic findings were consistent with a complex, multiseptated, hypoechoic mass. Two of the 3 patients underwent radical orchiectomy. Testis sparing mass excision was performed in 1 patient. Grossly the tumors were partially cystic masses. Histologically positive immunostaining with inhibin-alpha and negative staining for alpha-fetoprotein (AFP) reliably differentiated JGCTs from yolk sac tumors. At a mean followup of 8.5 years (range 5 to 14) no metastases or local tumor recurrences have been diagnosed. CONCLUSIONS: To our knowledge we report the first case of testis sparing enucleation of a JGCT with a 5-year recurrence-free followup. Testis sparing enucleation is now our procedure of choice for tumors in neonates and prepubertal children with serum AFP in the normal range for age. JGCT should be suspected in neonates presenting at birth with a complex, cystic mass of the testis. Positive immunostaining for inhibin-alpha and a lack of AFP staining have consistently corroborated the pathological diagnosis in our experience and they should be applied for pediatric testis tumors that may mimic yolk sac tumor pathology.     

Intratubular malignant germ cells (carcinoma in situ) accompanying invasive testicular germ cell tumors

We examined 111 radical orchiectomy specimens from patients with germ cell tumors of the testis treated between 1971 and 1981 for the presence of intratubular malignant germ cells (so-called carcinoma in situ) in adjacent testicular tissue. Of the specimens 12 were unevaluable because the entire specimen was tumor, while 36 of 44 cases of pure seminoma (82 per cent) and 41 of 55 mixed or nonseminomatous tumors (75 per cent) had intratubular malignant germ cells. Of the 22 cases without intratubular malignant germ cells 10 had severe atypia that suggested an earlier or borderline stage of development. These data support the contention that germ cell tumors originate as intratubular neoplasms or so-called carcinoma in situ in the seminiferous tubules.     

Conservative management of small testicular tumors relative to carcinoma in situ prevalence

PURPOSE: We evaluated the prevalence of carcinoma in situ (CIS) in orchiectomy specimens performed for germ cell tumors smaller than 40 mm in diameter to propose an appropriate conservative approach to bilateral tumors or tumor of a solitary testis. MATERIALS AND METHODS: Of 127 patients treated with orchiectomy between 1990 and 2002, 41 who presented with a tumor of less than 40 mm in diameter were selected for histological analysis of testicular parenchyma. The morphological items assessed were CIS, spermatogenesis and Leydig cell hyperplasia. RESULTS: CIS was observed in 39 of the 41 patients (95%). CIS was evenly distributed throughout the testicular parenchyma (ie around and beyond the tumor) in all 39 cases. Spermatogenesis was observed in 12 of 41 specimens (29%), spermatogenesis without spermatozoa was noted in 14 (34%) and absent germ cells were found in 15 (37%). Leydig cell hyperplasia was observed in 24 cases (58%). CONCLUSIONS: Histological analysis of whole orchiectomy specimens showed that CIS is almost always present in testicular parenchyma adjacent to germ cell tumor. In bilateral testis cancer or cancer occurring in a solitary testis tumorectomy plus radiotherapy appears to be the appropriate treatment in patients with a small tumor and no other risk factors. In patients who wish to father a child and have preserved spermatogenesis the natural history of CIS allows the postponement of testicular radiotherapy after orchiectomy, giving the double advantage of preserving testicular endocrine function and maintaining the possibility of natural fatherhood.     

Testis sparing surgery for the treatment of a sequential bilateral testicular germ cell tumor

Standard therapy of sequential bilateral testis cancer is generally considered to be orchiectomy. We present a case of sequential bilateral testicular germ cell tumor treated with testis sparing surgery. The patient was disease free 50 months after surgery without local recurrence or distant metastases. Testis sparing surgery provides a better quality of life and may be considered a safe, feasible alternative in the treatment of carefully selected patients with bilateral testicular germ cell tumor.     

Histology in mixed germ cell tumors. Is there a favorite pairing?

PURPOSE: Mixed germ cell tumors account for approximately 30% to 50% of testicular tumors. To our knowledge a systematic review with statistical analysis of the associations of histological subtypes in mixed germ cell tumors has not been done previously. It was our impression that such associations exist. Delineating concordant histological types may provide insight into the ontogeny of testicular tumors and also have important clinical implications. MATERIALS AND METHODS: We retrospectively reviewed the testis cancer data base at our institution. The primary tumor of orchiectomy specimens was examined in 2589 patients. Of these patients mixed histology was noted in 1765 (68.2%). ORs were calculated for all possible combinations of teratoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma and seminoma. In addition, we evaluated the association of various histological types with teratoma at post-chemotherapy retroperitoneal lymph node dissection. RESULTS: Of 10 possible combinations of histological types in the primary tumor, positive correlations were noted in 4. The strongest correlation was found between teratoma and yolk sac tumor (OR 2.58, p <0.001). Teratoma or yolk sac tumor in the testis was associated with teratoma in the pathology specimen at post-chemotherapy retroperitoneal lymph node dissection. CONCLUSIONS: The strongest associations of histological subtypes in mixed germ cell tumors were seen between yolk sac tumor and teratoma. Similar associations are seen in late relapse and in some cases of prepubertal tumors. Further study of these associations may prove valuable in understanding the biology and clinical behavior of germ cell tumors.     

The Role of Retroperitoneal Lymphadenectomy in Mature Teratoma of the Testis

Purpose

Pure testicular teratoma is rare in adulthood with an incidence of 5%. Pure teratoma is considered less aggressive and less likely to metastasize than other nonseminomatous germ cell tumors. Therefore, patients with mature teratoma have been considered candidates for surveillance protocols. We report our experience with 44 cases of primary pure testicular teratoma.

Materials and Methods

We retrospectively identified 44 patients (5.7%) with primary pure teratoma of the testis of the 772 treated for testicular germ cell tumors at our institutions. Archival tumor blocks were available for pathological reevaluation and serial sections were obtained in all cases. A total of 35 patients (79.5%) who presented with clinical low stage disease, including stage I in 26 (59.1%) and stage IIA/B in 9 (20.4%), underwent radical orchiectomy followed by retroperitoneal lymphadenectomy. Nine patients (20.5%) who presented with clinically advanced disease (stages IIC to IV) were treated with primary chemotherapy and secondary retroperitoneal lymphadenectomy of residual masses.

Results

The frequency of lymph node metastases was 19.2% in clinical stage I disease and 66% in stage IIA/B. Histopathological diagnosis of mature teratoma was confirmed in all cases. However, of 20 patients 16 (80%) had scars or calcifications in the adjacent parenchyma, indicating a burned out tumor, and 4 (20%) had microfocal embryonal carcinoma. None of the patients with clinical stage I disease had relapse during followup and the relapse rate in those with stage IIA/B disease was 33%. Median followup was 97 months (range 24 to 250). Overall 43% of patients with pure teratoma presented with metastatic disease.

Conclusions

Our data demonstrate the malignant potential of pure testicular mature teratoma. Based on our results metastases in testicular mature teratoma seem to result from metastasizing nongerm cell components undergoing early regression, as demonstrated by the high frequency of burned out tumors. We recommend that serial sections be taken of the orchiectomy specimen in all cases of pure mature teratoma to determine adequate management: retroperitoneal lymphadenectomy in cases of associated scars, calcifications or microfocal malignant germ cell components and surveillance in cases of pure mature teratoma.     

A case of organ sparing surgery for metachronous bilateral testicular tumor with maintaining testicular function

A 28-year-old man, who had undergone right orchiectomy and prophylactic irradiation for stage I seminoma 6 years ago, developed left testicular tumor. Since the secondary tumor was localized in the lower pole of the testis, partial orchiectomy was performed with an attempt to preserve the testicular function. The pathological finding of the surgical specimen was a mixed type testicular tumor consisting of seminoma, embryonal carcinoma and teratoma elements. Postoperative chemotherapy with 3 courses of BEP regimen resulted in azoospermia, but the impaired spermatogenesis recovered to a normal range within 18 months with no evidence of tumor recurrence and his wife delivered a healthy baby 2 years later. For the synchronous or metachronous bilateral testicular tumors, the combination of organ sparing surgery and chemotherapy could be a treatment of choice.     

Diagnosis and management of teratomas and epidermoid cysts

One can strongly suspect a benign lesion in a prepubertal child on the basis of preoperative sonographic, biochemical, and clinical evaluation; however, when considering a testis-sparing enucleation, the definitive diagnosis must be established by pathologic frozen section of the tumor. The high incidence of benign testicular lesions in the prepubertal patient, the absence of associated carcinoma in situ in prepubertal germ cell tumors, and the universally benign behavior of the specific tumors described in this article are supportive evidence for testis-sparing surgery versus orchiectomy, for these benign lesions.     

Prepubertal testicular tumours and efficacy of testicular preserving surgery

Study Type – Therapy (case series)
Level of Evidence 4 What’s known on the subject? and What does the study add? Testicular tumours in childhood are very rare. Historically, most of these tumours have been considered malignant, but more recent studies indicate that benign lesions, particularly teratoma, are much more frequent than previously thought. Testicular tumours in this age group have traditionally been treated with inguinal radical orchiectomy, but more conservative management has been proposed in view of the higher frequency of benign tumours. In children, most testicular tumours are benign, especially before puberty. A testis‐sparing procedure should be performed in children with a palpable testicular mass and negative tumour markers.

OBJECTIVE

? To report our experience of testicular tumours in children aged ≤13 years, including our experience with testis‐sparing surgery.

PATIENTS AND METHODS

? A retrospective study was performed of 15 patients with testicular tumours aged ≤13 years who presented at our centre between 1984 and 2008. The use of testis‐preserving surgery according to indication was investigated and outcomes were recorded.

RESULTS

? The clinical presentation was increased testicular size with a palpable mass in 80% of the cases. All 15 patients underwent surgery. The tumour was benign in 12 (80%) patients and malignant in three (20%) patients. ? Organ‐preserving surgery was planned and achieved in 11 patients (73%). ? Pathology of the tumourectomy specimens disclosed benign tumours in all cases: four epidermoid cysts, two teratomas, one juvenile granulosa cell tumour, one haemangioma, one lipoma, one fibrous hamartoma and one splenogonadal fusion. ? In four patients who underwent radical orchiectomy, pathology identified one yolk sac tumour (stage I), two mixed germ cell tumours and one gonadoblastoma.

CONCLUSIONS

? In children, most testicular tumours are benign, especially before puberty. A testis‐sparing procedure should be performed in children with a palpable testicular mass and negative tumour markers. ? The lesion, however, should be thoroughly excised to avoid recurrences.     

Cystic testicular lesions in the pediatric population

PURPOSE: We present the etiology, histological evaluation and management of all cystic lesions of the pediatric testis. MATERIALS AND METHODS: Illustrative cases from our experience are reported with a literature review of all possible diagnoses. RESULTS: Included in the differential diagnosis of cystic testis lesions in children are epidermoid cyst, dermoid cyst, prepubertal teratoma, juvenile granulosa cell tumor, cystic dysplasia of the rete testis, testicular cystic lymphangioma, simple cyst and cystic degeneration after torsion. Testis sparing surgery is feasible in many circumstances. CONCLUSIONS: Cystic lesions of the pediatric testis are rare but represent an interesting group of diagnoses. Patient age at presentation, examination features, tumor markers and sonographic appearance may assist in making a presumptive and occasionally definitive diagnosis preoperatively. Based on the likely diagnosis enucleation or partial orchiectomy may be considered when performed with frozen section histological assessment. A thorough understanding of potentially cystic testis lesions in children leads to the best management choices and often to preservation of a substantial portion of the affected testis.     

Testis Sparing Surgery for Infantile Synchronous Bilateral Teratoma of the Testis

We report a rare case of infantile synchronous bilateral testicular teratoma treated by testis sparing surgery. Preoperative ultrasonography, tumor marker status and intraoperative findings were suggestive of benign neoplasia. Under these circumstances we performed high orchiectomy for the left testis and testis sparing surgery for the right. Surgical treatment of teratoma in childhood is controversial, especially when it occurs bilaterally. In this report we discuss testis sparing surgery as the treatment for this disease.     

Serum lactate dehydrogenase and its isoenzymes in men with maldescended testes

Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were determined as a screen for testicular germ cell neoplasia in 130 men with maldescended testes. A testicular tumor was found on clinical examination in 1 patient, which was revealed to be embryonal carcinoma, teratoma, yolk sac tumor and carcinoma in situ on orchiectomy. Subclinical testicular germ cell neoplasia was found on testicular biopsy in 3 men (1 with microinvasive seminoma and 2 with carcinoma in situ). These 4 patients had normal serum lactate dehydrogenase and serum lactate dehydrogenase isoenzymes. Elevated serum lactate dehydrogenase was noted in 3 men without testicular germ cell neoplasia: 1 had predominantly increased serum lactate dehydrogenase isoenzymes 1 to 3 and 2 had slightly increased serum lactate dehydrogenase isoenzymes 3 and 4. Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were not sensitive to detect testicular germ cell tumors in a subclinical stage.     

Testicular and paratesticular prepuberal tumors: our experience and update on the topic

Objetivesto evaluate the importance of testicular and paratesticular prepubertal tumors in our center and to make an update on the topic.Methods and patientsdata from all patients diagnosed of testicular and paratesticular prepubertal tumors and treated in our pediatric oncology unit from January 1^st 1998 to December 31^st 2003 have been revised.Resultsseven cases are reported among one hundred and ninety patients (represents 3,68 percent of all treated tumors): five tumors affecting the testis and two cases of paratesticular tumors. Pathology classification was as follows: one yolk salk tumor, one mature teratoma, two nongerminomatous testicular tumors (one Sertoli cell tumor and one unclassifiable), one Burkitt’s lymphoma and two paratesticular rhabdomyosarcomas. Primary approach was inguinal radical orchiectomy in all cases except neoadjuvant chemotherapy in the case of lymphoma and partial escrotectomy in one patient previously managed with transcrotal orchiectomy. Rhabdomyosarcoma cases received adjuvant chemotherapy. All patients are alive and well after a follow-up period ranging from 17 to 74 months.Conclusionstesticular and paratesticular prepubertal tumors are rare. Except for one patient affected of lymphoma, surgical primary approach have been essential for treatment. The prognoses in this series has been excellent.     

Recurrent rhabdomyosarcoma after adjuvant chemotherapy for stage I non‐seminomatous germ cell tumor with malignant transformation

We report a rare case of stage I non‐seminomatous testicular germ cell tumor with malignant transformation. The patient received two cycles of chemotherapy (cisplatin, bleomycin and etoposide) tailored to testicular germ cell tumors as an adjuvant therapy after orchiectomy. However, 22 months later, the patient developed a metastasis in the occipital region that consisted of solely rhabdomyosarcoma through malignant transformation of a teratoma component. This case highlights an issue related to adjuvant chemotherapy for testicular germ cell tumors with components of malignant transformation.     

Signet ring cell carcinoma of the testis: clinicopathologic and molecular evidence for germ cell tumor origin--a case report

Development of a somatic-type malignancy from a mixed germ cell tumor is a rare but recognized event and typically represented by sarcoma or, less commonly, by carcinoma. This phenomenon is generally believed to result from progression of a teratomatous component. In many cases, because of intermingling of other germ cell tumor components, the diagnosis is apparent; however, in rare cases, metastatic carcinoma to the testis or a novel primary tumor may be a diagnostic consideration. In this study, we report the clinicopathologic, immunohistochemical, and molecular features of a 53-year-old man, whose testicular tumor was composed entirely of signet ring cells, mimicking metastatic carcinoma. Subsequent retroperitoneal lymph node dissection revealed metastatic deposits composed of teratoma and yolk sac tumor, in addition to signet ring cell carcinoma. Fluorescence in situ hybridization for abnormalities of chromosome 12p revealed the presence of i(12p) in both the teratoma and signet ring cell carcinoma in the metastasis and in signet ring cells in the testis, supporting a common germ cell origin. Our report indicates that signet ring carcinoma cells in an orchiectomy specimen, although usually strongly suggestive of metastatic adenocarcinoma from a primary tumor in another organ, may be a primary testicular neoplasm of germ cell tumor origin. This is the first report of testicular signet ring cell carcinoma of germ cell tumor derivation.     

Prepubertal testicular tumors: A 20‐year experience with 40 cases

Testicular tumors are rare in children. In addition, prepubertal testicular tumors (PTT) are fundamentally different from their postpubertal counterparts. We reviewed our 20‐year experience with 40 cases to suggest an algorithm for the appropriate surgical approach for patients with PTT. All tumors ultrasonographically suspected of being benign were confirmed to be benign postoperatively. There was a significant difference between median preoperative α‐fetoprotein (AFP) levels of infants with yolk sac tumor and teratoma. The majority of our cases were benign, with the most common histopathological subtype being teratoma, as recently reported. Testis‐sparing surgery was carried out in five patients with teratoma and three patients with epidermoid cyst. Other than one patient with teratoma, none of the patients developed recurrence or testicular atrophy after testis‐sparing surgery. When testis‐sparing surgery is considered, our algorithm based on ultrasonographic findings and preoperative AFP levels might be helpful.