A Rare Case of a Testicular Teratoma Associated with a Neuroendocrine Tumour.

Introduction: We report a rare case of testicular teratoma combined with a neuroendocrine tumour, emphasizing the difficulty of the following aspects: the clinical and laboratory diagnosis, the treatment options and the evolution of patients suffering from this disease. Case presentation: The patients with testicular neuroendocrine tumours represent a rarity, considering that as of 2017, only 22 cases had been reported in the literature. The case operated on in our clinic presents an association between a testicular teratoma and a neuroendocrine tumour. A 39-year-old patient was admitted in our Department for a non-painful abdominal tumour and concomitant testicular tumour. The serum tumour markers (-human chorionic gonadotropin, -phetoprotein and lactate dehydrogenase) were within normal limits. Lung and bone metastases were diagnosed CT scan. The histopathological diagnosis consisted of immunohistochemical study of the orchidectomy specimen as well as of the bioptic material from bone marrow puncture. Conclusions: The diagnosis of testicular carcinoids is based on immunohistochemistry study. Radical orchidectomy is the only potentially curative treatment for this type of malignancy. Adjuvant chemotherapy determined size reduction of the lung and bone metastases and the disappearance of the lymph node metastases.

Peritoneal Carcinomatosis of Rare Ovarian Origin Treated by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: A Multi-Institutional Cohort from PSOGI and BIG-RENAPE.

PURPOSE: Ovarian cancer is the most common deadly cancer of gynecologic origin. Patients often are diagnosed at advanced stage with peritoneal metastasis. There are many rare histologies of ovarian cancer; some have outcomes worse than serous ovarian cancer. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) can be considered for patients with recurrence. This study was designed to assess the impact of CRS and HIPEC on survival of patient with peritoneal metastasis from rare ovarian malignancy. METHODS: A prospective, multicentric, international database was retrospectively searched to identify all patients with rare ovarian tumor (mucinous, clear cells, endometrioid, small cell hypercalcemic, and other) and peritoneal metastasis who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI) and BIG-RENAPE working group. The postoperative complications, long-term results, and principal prognostic factors were analyzed. RESULTS: The analysis included 210 patients with a median follow-up of 43.5 months. Median overall survival (OS) was 69.3 months, and the 5-year OS was 57.7%. For mucinous tumors, median OS and DFS were not reached at 5 years. For granulosa tumors, median overall survival was not reached at 5 years, and median DFS was 34.6 months. Teratoma or germinal tumor showed median overall survival and DFS that were not reached at 5 years. Differences in OS were not statistically significant between histologies (p = 0.383), whereas differences in DFS were (p < 0.001). CONCLUSIONS: CRS and HIPEC may increases long-term survival in selected patients with peritoneal metastasis from rare ovarian tumors especially in mucinous, granulosa, or teratoma histological subtypes.

Management of patients with metastatic teratoma with malignant somatic transformation.

PURPOSE OF REVIEW: The purpose of this review is to examine the historical context alongside contemporary studies in order to provide the most current recommendations for the management of patients with metastatic teratoma with malignant somatic transformation (MST). RECENT FINDINGS: The main themes in the recent literature covered herein include prognostic features, the management of early-stage disease, recommended chemotherapeutic and surgical strategies as well as recognized patterns of late relapse. SUMMARY: Recent literature, combined with a significant contribution from historical studies, suggests that while MST is uncommon, its aggressive nature coupled with its resistance with traditional germ cell tumor chemotherapies makes it very difficult to manage. The key message is that surgery is recommended in all resectable MST from primary retroperitoneal lymph node dissection for clinical stage I, to radical removal of disease after chemotherapy and when chemotherapy fails. In advanced cases with documented spread of the transformed histologic subtype, systemic therapies targeted to the identified tumor type should be considered.

[Retroperitoneal metastasis from testicular tumor: Mature teratoma with adenocarcinomatous transformation]. / Métastase rétropéritonéale d'une tumeur testiculaire sous la forme d'un tératome mature avec transformation en adénocarcinome.

A 41-year-old patient, with a medical history of embryonal carcinoma treated by orchidectomy at the age of 27, presented with abdominal pain. Serum alphafetoprotein, ß-HCG and CEA levels were normal. MRI showed a cystic retroperitoneal lesion with septation, measuring 20cm. He underwent a surgical resection. Histology revealed an adenocarcinoma arising on a mature multitissular teratoma.

Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study.

BACKGROUND: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. METHODS: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. RESULTS: In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9-80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2-165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate ( p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78-3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. CONCLUSIONS: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.

Growing teratoma syndrome after ovarian inmature teratoma: a case report and review of the literature.

Growing teratoma syndrome is an uncommon complication of malignant germ cell cancer, characterised by the development of large tumours during or after chemotherapy, despite normalisation of tumour marker levels and metastasis, which contain only mature teratoma. Given its low incidence, little is data available. The authors present the case of a 15-year-old girl with a growing teratoma and the literature review outlines the current knowledge of its pathogenesis, common sites, diagnosis, natural course, treatment, and prognosis.

Cutaneous location of atypical teratoid/rhabdoid tumour.

Atypical teratoid/rhabdoid tumour is a rare and highly malignant tumour of the posterior fossae nervous system that occurs in children especially in the first few years of life. Cutaneous location is not previously reported. A newborn boy was referred for both aqueductal stenosis detected antenatally and skin tags mimicking hamartoma. The cerebral tumour increased in size during a few months leading to both skin and cerebral biopsies. Integrase Interactor-1 (INI-1) immunostaining and tumoural and leukocytes INI-1 gene sequencing confirmed the atypical teratoid/rhabdoid tumour nature of the cerebral tumour. INI-1 immunostaining in skin biopsy confirmed the dermal location of rhabdoid tumour. Thus, unusual cutaneous lesions may be part of atypical teratoid/rhabdoid tumour. The loss of Integrase INI-1 on immunohistochemical staining is characteristic.

Growing teratoma syndrome in intracranial non-germinomatous germ cell tumors (iNGGCTs): a risk for secondary malignant transformation­a report of two cases.

PURPOSE: About 5% of pediatric intracranial germ cell tumors and 20% of non-germinomatous germ cell tumors (NGGCT) progress to growing teratoma syndrome (GTS) following chemoradiotherapy. The growing teratoma is thought to arise from the chemotherapy-resistant, teratomatous portion of a germ cell tumor and is commonly benign but may undergo malignant transformation. METHODS: Two pediatric patients whose intracranial NGGCTs progressed to growing teratomas during chemotherapy and later transformed to secondary malignant tumors after partial resection and radiation therapy (RT). RESULTS: Both tumors were diagnosed by MRI scans and elevated serum and CSF markers. Following normalization of tumor markers with chemotherapy and initial decrease in tumor volume, subsequent imaging showed regrowth during chemotherapy with pathology revealing benign teratoma. RT was administered. Several years following this treatment, further growth was seen with pathology indicating malignant carcinoma in one patient and malignant rhabdomyosarcoma in the other. The patient with carcinoma received palliative care while the patient with the sarcoma received further resection, intensive chemotherapy, and an autologous stem cell transplant and is currently in remission, 36 months since malignant transformation. CONCLUSION: Malignant transformation of presumed residual teratoma has been seldom reported. Treatment of NGGCT involves platinum-based chemotherapy with craniospinal RT and boost to the primary site, with cure rates of around 80%. Teratomas are characteristically chemotherapy and RT resistant and are treated surgically. In the event that residual or growing teratoma is suspected, a complete resection should be considered early in the management as there is a risk of malignant transformation of residual teratoma.

Clinical and pathological features predictive of nephrectomy at post-chemotherapy retroperitoneal lymph node dissection.

PURPOSE: We determined the clinical and pathological features associated with nephrectomy at post-chemotherapy retroperitoneal lymph node dissection. MATERIALS AND METHODS: We retrospectively reviewed the testis cancer database from 1980 to 2007 to identify all patients treated with post-chemotherapy retroperitoneal lymph node dissection. Patients with pure seminoma and nongerm cell histology were excluded from study. A total of 1,807 patients were identified, of whom 17 without recorded mass size were excluded from further study. Pathological and clinical variables were assessed by bivariate analysis. Multivariate logistic regression was used to determine predictors of nephrectomy at post-chemotherapy retroperitoneal lymph node dissection. RESULTS: The overall incidence of nephrectomy at post-chemotherapy retroperitoneal lymph node dissection was 14.8% (265 of 1,790 cases). The incidence of nephrectomy was 17.0%, 18.9%, 13.6% and 8.0% in 1980 to 1988 (group 1), 1989 to 1997 (group 2), 1998 to 2002 (group 3) and 2002 to 2007 (group 4) (p = 0.0001). The nephrectomy rate for tumors less than 2, 2 to 5, 5 to 10 and greater than 10 cm was 6.0%, 5.8%, 13.9% and 31.9%, respectively (p = 0.0001). The incidence of nephrectomy based on retroperitoneal histology was 10.3% for fibrosis, 14.5% for teratoma and 20.4% for cancer (p = 0.0001). The strongest predictor of nephrectomy at post-chemotherapy retroperitoneal lymph node dissection was retroperitoneal mass size greater than 10 cm (OR 9.30, 95% CI 3.8-22.7). CONCLUSIONS: The incidence of nephrectomy at post-chemotherapy retroperitoneal lymph node dissection has decreased in the last 3 decades. A higher incidence was observed in patients with larger volume tumors, those who received salvage chemotherapy, those with a left primary testicular tumor and those with increased markers at post-chemotherapy surgery.

Retroperitoneal teratoma with somatic malignant transformation: a papillary renal cell carcinoma in a testicular germ cell tumour metastasis following platinum-based chemotherapy.

BACKGROUND: Malignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation. CASE PRESENTATION: A 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed.Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histo-pathological evidence of a primary renal cell cancer. CONCLUSIONS: To the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens.

["Growing teratoma syndrome": an unrecognized complication of treated germ cell tumors of the testis]. / Le « growing teratoma syndrome ¼ : une évolution méconnue des tumeurs germinales non séminomateuses traitées du testicule.

"Growing teratoma syndrome" is a rare and often unrecognized complication of nonseminomatous germ cell tumors of the testis. It is defined by enlarging residual masses, frequently retroperitonal, composed exclusively by teratoma, during the course of chemotherapy. Complications of this syndrome are due to masses compression. Malignant transformation is also possible. "Growing teratoma syndrome" has a good prognosis when cured by complete surgical excision of the tumoral masses. We report the case of a "growing teratoma syndrome" presenting as a retroperitoneal mass occurring in a patient previously treated by orchiectomy and chemotherapy for a nonseminomatous mixed germ cell tumors of the testis without teratomatous component.

[A case of mediastinum malignant teratoma which recurred as bone metastases seven years after a resection, and survived for a long-duration].

We performed median sternotomy, anterior mediastinum tumor resection+cardiac sac, and right pleural+superior vena cava partial excision in the anterior mediastinum, for which tumor markers were abnormal, after preoperative chemotherapy (PEB therapy)for a mediastinal malignant teratoma of 8×6 cm in a malignant germ cell tumor. The tumor markers became normal. We postoperatively performed a course of the same chemotherapy. In spite of the normal tumor markers, bone metastases recurred seven years later, and he underwent the ninth thoracic vertebra resection+spinal fusion. Five years have passed since surgery and he has survived without a recurrence. This seemed to be a rare case in which a part of the mediastinum malignant teratoma which did not produce a tumor marker had a recurrence.

A rare case of solitary metastatic non-seminomatous malignant germ cell tumor to the prostate.

Testicular cancer is the most common solid malignancy of men aged 15-40 years and metastasizes in a predictable manner via lymphatic spread. Involvement of metastatic testicular cancer to the prostate is an exceedingly rare event which has only been previously described in patients with seminomatous germ cell tumors. In this report, we present a case of a 42-year-old man who presented with metastatic testicular cancer to the prostate 8 years after his original diagnosis of a mixed germ cell left testicular tumor.

Large teratoma at aortic bifurcation: an unusual metastasis of testicular cancer.

A 29-year-old man presented with a right testicular mass. Serum tumor markers were within normal limits. When compared to a previous computed tomography (CT) scan, a new 4 cm presacral mass was present. He underwent radical right inguinal orchiectomy that demonstrated a mature teratoma and seminomatous components. The patient received four cycles of chemotherapy. Over the course of chemotherapy, the mass grew in size and therefore he underwent retroperitoneal lymph node dissection. Pathology confirmed it to be a teratoma with negative retroperitoneal lymph nodes. The unusual presentation of an isolated metastasis to the presacral region raises the question of altered lymphatic drainage.

Primary ovarian teratoma and GCT with intra-abdominal metastasis in a dog.

This report describes the simultaneous occurrence of an ovarian teratoma and a granulosa cell tumor (GCT) with intra-abdominal metastasis in a 1.5 yr old female Doberman pinscher. At surgery, a 20 cm, smooth, intact mass associated with the left ovary and multiple 1-2 cm irregular masses in the broad ligament were found. The masses were surgically removed and submitted for histopathology. A histologic diagnosis of a teratoma and a GCT with broad ligament metastasis was made. Further treatment was elected by the owner and included two cycles of carboplatin therapy. The dog was euthanized 6 wk postoperatively for signs related to metastasis and dyspnea. Teratoma of the ovary, although it contains derivatives of all three embryonic germ cell layers, rarely presents together with either ovarian epithelial or sex cord-stromal tumors. To the authors' knowledge, this is the first reported case of an ovarian teratoma coexisting with a primary GCT with intra-abdominal metastasis in the same ovary in a dog.

Retroperitoneal lymph node dissection with concomitant IVC thrombectomy, caval wall resection, and grafting for metastatic NSGCT.

INTRODUCTION: The management of a post-chemotherapy retroperitoneal mass secondary to testicular cancer can present a surgical challenge when involving adjacent organs or major vascular structures. We present the first video of a retroperitoneal lymph node dissection (RPLND) with IVC (inferior vena cava) thrombectomy, caval wall resection resulting from metastatic non-seminomatous germ cell testis (NSGCT) cancer. METHODS: In this surgical video, we highlight important surgical considerations in the management of a postchemotherapy retroperitoneal mass with direct IVC wall invasion and level 2 thrombus in such a patient. RESULTS: A 34 year old man underwent a right inguinal orchiectomy for a mixed NSGCT (embryonal, yolk sac, and teratoma components) and elevated serum tumor markers. He underwent systemic chemotherapy (BEP regimen x 4 cycles) with subsequent near normalization of tumor markers. His post-chemotherapy imaging revealed a 6 cm residual retroperitoneal mass with a level 2 IVC tumor thrombus and suspected direct infrarenal IVC wall invasion from the mass. The patient underwent an open post-chemotherapy RPLND, IVC thrombectomy, IVC resection and grafting. The final pathology report of the retroperitoneal mass revealed teratoma with no viable germ cell tumor elements and negative surgical margins. His intra-operative and post-operative stages were unremarkable with his IVC graft remaining patent and no evidence of disease recurrence at last follow-up. CONCLUSION: We present the first surgical video of a post-chemotherapy RPLND with IVC thrombectomy, caval wall resection and grafting for metastatic NSGCT. The final pathology report of teratoma with no viable tumor highlights the local vascular invasive potential of such pathology.

[Gastric metastases with a choriocarcinoma component from a postpuberal teratoma with mature histology]. / Metástasis gástricas con componente de coriocarcinoma de un teratoma testicular pospuberal con histología madura.

Germ cell tumors are the most frequent neoplasia in young males. The aims of this study is to describe a case in which a postpuberal teratoma suffers a transformation to choriocarcinoma and metastasize to stomach. We have made a systematic review in PubMed and consensus documents to study this mismatch between the tumour, metastasis and the exception of gastric metastatic affectation. We describe three options to explain this discordance: a mixed germ cells tumour, a burned out tumour or a germ cells tumour derived from a malignant germ cell tumour precursor or different clonal strains. After made a thorough investigation we conclude that the most truly option is the last one as we extensive explain below. Once the gastric metastatic lesions are extremely rare and reach to <5%, but there are not conclusive assessments.

Towards the definition of the best management and prognostic factors of teratoma with malignant transformation: a single-institution case series and new proposal.

OBJECTIVE: • To investigate the optimal management and prognostic factors of patients with malignant transformation (MT) in germ-cell tumour (GCT) by re-evaluating Institutional series. PATIENTS AND METHODS: • Patients with an MT within GCT have been identified from the institutional database and all slides have been reviewed by the referral pathologist. RESULTS: • From June 1982 to October 2009, 48 patients and 13 somatic histologies have been identified. Twelve patients presented with stage I, 12 with stage II and 24 with stage III disease. All stage I patients are alive and disease-free after a median follow up of 88 months (interquartile range 38-103). • Of the 36 metastatic cases, 11 underwent GCT-oriented chemotherapy plus surgery and seven of them are currently disease-free. Three patients underwent MT-chemotherapy, one relapsed and is still under treatment. Overall, 17 patients relapsed (35%) and three of them have been rescued by GCT-chemotherapy. Five-year overall survival was 100% for stage I, 80% (95% CI 40-94) for stage II and 44% (95% CI 19-67) for stage III patients. Stage III disease at MT, incomplete surgical removal and primitive neuroectodermal tumours plus adenocarcinoma histologies were significant adverse prognostic factors for survival. CONCLUSIONS: • New insights emerged into the impact of histology and chemotherapy on MT. The development of an adenocarcinoma component as well as the possible efficacy of a GCT-tailored chemotherapy in a multimodal strategy are addressed for the first time, while disease extent at transformation and extent of radical surgery are confirmed as significant prognosticators. • An international web database for registration of all cases of MT worldwide is presented.

Soft tissue metastasis of an immature teratoma of the ovary.

In patients with immature teratoma of the ovary, blood-borne metastasis to organ parenchyma, such as the lungs, liver, or brain, are uncommon. Moreover, soft tissue metastasis is extremely rare. We describe a 31-year-old woman with an immature teratoma of the left ovary, which metastasized to soft tissue of the right thigh after surgery. Because of the rarity of this condition, we report the results of treatment and review the literature.