Teratoma arising from anomalous common bile ducts: a case report

Teratoma arising from extrahepatic common ducts is very rare entity. The authors found 2 teratoma cases originating from common bile duct in the literature. As a third case, the authors report on a 4-month-old girl with benign cystic teratoma arising from distal common hepatic bile duct and with anomalous common bile ducts. Surgical management of the patient also is discussed.     

Congenital teratoma: a clinicopathologic study of 22 fetal and neonatal tumors

Extragonadal teratoma is the most common congenital tumor. The prognostic significance of the grade of immaturity and the presence of small foci of conventional yolk sac tumor (YST) in fetal and neonatal teratomas have not been determined. We report detailed histologic studies of 22 congenital teratomas, including eight tumors resected in utero for developing hydrops, and correlate the histologic features with initial serum alpha-fetoprotein (AFP) levels and clinical outcome. All fetal tumors that required in utero intervention were grade 3 immature teratomas, with admixed conventional YST in 44%. Among tumors resected postnatally, those presenting in utero were more commonly immature (71% vs. 50%). All initial post-surgical serum AFP levels were high, as expected in a neonate. No correlation was found between AFP elevation above the mean for gestational age and the presence of YST, hepatic differentiation, or immature endodermal glands in the tumor. Among 15 survivors with follow-up, 5 patients had malignant mixed germ cell tumors (immature teratoma with foci of conventional YST) and 5 had immature teratomas with foci of hepatic differentiation or immature endodermal glands with subnuclear vacuoles (so-called "well-differentiated YST"). No patient has developed recurrent or metastatic disease after treatment by complete surgical excision alone (mean follow-up, 37.6 months). The clinical behavior of congenital teratomas is determined predominantly by whether or not the tumor can be completely resected and in our study did not correlate with the grade of the teratoma or with the presence or absence of foci of hepatic tissue, immature intestinal glands, or foci of conventional YST.     

Chemotherapy for poor risk germ cell tumours. An independent evaluation of the POMB/ACE regime

We have evaluated the 7-drug, alternating, high-dose cisplatin regime for germ cell tumours, designated POMB/ACE, in 55 patients with advanced malignant teratomas and 5 patients with bulky metastatic seminomas. All of the latter and 5 of the teratoma patients had relapsed following radiotherapy, chemotherapy or both. The previously untreated teratoma patients included 13 whose tumours were extragonadal. The primary testicular tumour patients comprised 16 with large and 21 with very large volume metastases according to the Medical Research Council criteria. POMB/ACE is effective therapy for poor risk patients with germ cell tumours (including those with the most advanced disease, i.e. hepatic and cerebral metastases) and prolonged treatment after marker normality seems unnecessary. It is a complex regime with significant toxicity and cannot be recommended for the treatment of patients with germ cell tumours who have an excellent prognosis with simpler, shorter and less toxic treatment.     

Hepatic Metastasectomy for Testicular Germ Cell Tumors: Is it worth it?

Background  Chemotherapy is highly effective for metastatic germ cell tumor (GCT), but experience with resection of hepatic metastases from GCT is limited. Methods  Fifteen patients with GCT metastatic to the liver underwent 16 hepatic operations (1975–2002). Pre-resection therapy, surgical pathology, and operative outcomes were reviewed. All patients were followed to death or last contact for survival and disease status. Results  Patients underwent biopsy (three), wedge resection (nine), bisegmentectomy (two), and major lobectomy (two). Hepatic histology included: necrosis (33%), viable tumor (27%), mature teratoma (13%), and benign histology (27%). Concomitant resection of extrahepatic disease (14 patients, 93%) found necrosis (53%), mature teratoma (27%), and viable tumor (13%). Operative mortality was 0% and morbidity was 40%. At 8.2 years (mean) from resection, 11 patients (73%) were alive: five with no evidence of disease, two with elevated tumor marker only, and four with gross disease. Four patients (27%) died. The 10-year overall survival was 62% from diagnosis. Conclusion  Resection of post-chemotherapy hepatic disease is safe, even when combined with resection of extrahepatic residual disease. The varied histologic findings, lack of reliable predictors, and prolonged survival achieved support a multidisciplinary approach which includes surgical resection of hepatic metastases. Presented at Digestive Disease Week, May 2008, San Diego, California     

Mediastinal mature teratoma perforating into the lung with elevated serum carbohydrate antigen 19-9 (CA19-9) levels; report of a case

A 17-year-old woman was suddenly seized with anterior chest pain and admitted to our hospital. Chest X-ray, computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mass shadow in the left middle lung field associated with the left pleural effusion and high serum carbohydrate antigen 19-9 (CA 19-9) levels (58.5 U/ml). We performed a total resection of the tumor with adherent parts of the left lung. The tumor was 6.5 x 3. 8 x 2.9 cm in size, and was made up of soft tissues, fluid and cystic components. The histological diagnosis was a mature cystic teratoma with perforation into the lung, which contained pancreatic tissues, hepatic cells, bronchial epithelium, serous glands and so on. The levels of fluid amylase and CA 19-9 were high. We report mediastinal mature teratoma perforating into the lung and discuss the mechanism of perforation.     

肾上腺畸胎瘤1例报告并文献复习

目的探讨肾上腺畸胎瘤的临床特点。方法分析我院收治的1例肾上腺畸胎瘤患者的临床资料及诊治方法,并复习国内外相关文献。结果患者为45岁女性,右腰背隐痛3 d就诊;B 超示肾上腺恶性肿瘤;CT 示畸胎瘤或错构瘤可能;术后病理证实为成熟性畸胎瘤。随访1年,未见局部转移或远处复发征象。结论肾上腺畸胎瘤是较罕见的生殖细胞来源肿瘤,临床症状及体征不典型,影像学检查是诊断的重要依据,病理检查是确诊的根本依据,应尽早手术切除;成熟畸胎瘤手术切除预后良好,未成熟畸胎瘤术后易复发,需化疗辅助治疗,且终身随访。     

Case report of a newborn with a posterior thoracic midline congenital hemangiopericytoma of the back

The posterior thoracic midline location is an unusual site for a congenital hemangiopericytoma. The authors report such a case that caused near fatal exsanguination of a newborn after vaginal delivery. Magnetic resonance imaging (MRI) studies of the mass were completed after hemostasis. These studies showed a well-defined border between the tumor and underlying trapezius muscle. The mass was removed successfully surgically and presumed initially to be a teratoma. Pathological diagnosis of the tumor was hemangiopericytoma with low malignant potential. After a 9-day hospital course, the patient was discharged with recovering hepatic and renal function.     

Histology of tumor residuals following chemotherapy in patients with advanced nonseminomatous testicular cancer

A total of 111 patients with advanced nonseminomatous testicular cancer underwent cisplatin-based combination chemotherapy, followed by surgical removal of residual masses in 101. Surgery included retroperitoneal lymph node dissection in 92 patients, thoracotomy in 19 and hepatic resection in 1 (11 patients underwent 2 operations). Complete necrosis and/or fibrosis was found in 52 operative specimens, mature teratoma in 37 and vital malignant tumor in 12. Of the 11 patients who underwent 2 operations 4 had complete necrosis and/or fibrosis in both histological specimens. After a median observation of 55 months 83 of 89 patients with complete necrosis and/or fibrosis or mature teratoma were without evidence of disease. Only 7 of 12 patients with vital malignant tumor in the operative specimen survived without evidence of disease. Relapses were observed in 16 patients, 4 of them in the retroperitoneal space. Of the 16 relapses 5 were in 12 patients with residual vital malignant tumor, 5 in 37 patients with post-chemotherapy mature teratoma and 4 in 52 patients with complete necrosis and/or fibrosis after chemotherapy. Two patients with recurrence did not undergo an operation. In patients in whom post-chemotherapy retroperitoneal lymph node dissection is considered complete necrosis and/or fibrosis can be predicted by the combination of several factors, including absence of teratomatous elements in the testicular tumor, complete response on post-chemotherapy computerized tomography, and normal alpha-fetoprotein and human chorionic gonadotropin levels after chemotherapy (sensitivity 83%, specificity 76% and correctly predicted 79%). With the knowledge of these factors it seems possible to omit post-chemotherapy retroperitoneal lymph node dissection in approximately 20% of the patients with advanced metastatic nonseminomatous testicular cancer with initial retroperitoneal tumors.     

Extraordinary large primary retroperitoneal cystic teratoma: An extremely rare neoplasm

Teratomas are the germ cell tumors which comprises of tissues from all the three germ cell layers. Primary retroperitoneal teratoma is a relatively rare tumor in adults. The primary retroperitoneal teratoma constitutes 6–11% of the retroperitoneal tumors. Sixty percent of the retroperitoneal teratomas occur in children less than 15 years.It occurs more commonly in females than males. The order of frequency for teratoma is ovary, testis, mediastinum and the retroperitoneum at last. Early diagnosis and surgery are the mainstay of treatment. We report a rare case of large primary retroperitoneal teratoma. This is the largest primary retroperitoneal teratoma reported in the literature to our knowledge.     

The management of metastatic germ cell tumours and the clinical utility of lactate dehydrogenase estimations

Forty-five patients with metastatic germ cell tumour were treated with chemotherapy. Complete remission was achieved in 63% of all cases and in 65% of patients whose primary tumour arose in the testis or ovary. Surgical resection of abdominal masses persisting after chemotherapy was performed in seven patients, two of whom were found to have persistent tumours. Twenty-seven of the 33 patients with teratoma originating in the gonads remain in complete remission. Total serum LDH activity was elevated in 28 of the patients with measurable disease. The increased LDH was not accompanied by significant alteration in other hepatic enzymes nor were hepatic metastases demonstrable in these patients. Fractionation of the LDH demonstrated that the increased LDH in these patients was located in either iso-enzymes 1 or fractions 1 + 2. Alteration of the serum LDH activity correlated with the response to therapy and warrants further study.     

A case of benign pulmonary metastasis from ovarian immature teratoma

A thirty one-year-old female underwent resection for pulmonary metastatic tumor from immature ovarian teratoma (G-II). Resected specimens from the lung revealed no malignant findings in the cystic teratoma (G-0). We speculated this finding might be due to retroconversion of ovarian immature teratoma. Retroconversion of ovarian immature teratoma is rarely reported and no report was found which dealt with pulmonary metastatic lesion histologically.     

Primary vs. post-chemotherapy retroperitoneal lymph node dissection (RPLND) in patients with presence of teratoma at orchiectomy

ObjectiveThe presence of teratoma in the primary orchiectomy specimen creates controversies for subsequent management. Although predominant teratoma is less likely to metastasize, teratoma in the retroperitoneum may be less amenable to chemotherapy. In order to elucidate the issues about teratoma in the primary tumor, we reviewed differences between primary retroperitoneal lymph node dissection (P-RPLND) vs. post-chemotherapy RPLND (PC-RPLND) in patients with teratoma at orchiectomy.Materials and methodsPatients who had undergone RPLND at our institution from 2001 to 2008 were identified, and clinical charts reviewed. Eighty-three patients with teratoma at orchiectomy were identified and perioperative data were obtained.ResultsOf the 83 patients with teratoma at orchiectomy who underwent RPLND, 44 (53%) and 39 (47%) underwent primary and PC-RPLND, respectively. Median follow-up was 1.4 years. Of the 83 patients with primary teratoma at orchiectomy, there were 7 (8%) patients with pure teratoma and 76 (92%) patients with mixed histology. Of the patients with mixed histology, 72 (87%) patients had embryonal carcinoma and 36 (43%) had LVI. There were 19 (43%) positive lymph nodes for P-RPLND, of which 13 (30%) contained teratoma. For the PC-RPLND group, 30 (77%) of lymph nodes were positive, of which 28 (72%) contained teratoma. There were 3 (4%) recurrences overall, all of which recurred in the PC-RPLND group. There were 11 (13%) perioperative complications total. There were no deaths in either group.ConclusionsPatients with teratoma at orchiectomy were associated with other high risk features and are at significant risk for metastatic disease. Patients with post-chemotherapy retroperitoneal findings are at significant risk for viable GCT and/or teratoma and should undergo PC-RPLND.     

Malignant transformation in a mature testicular teratoma left untreated for more than 50 years since childhood

Although testicular teratoma in childhood is regarded as a benign tumor, little is known about the consequences of pediatric teratoma being left untreated. We report herein a case of malignant transformation observed in a mature testicular teratoma that was presumed to have remained benign for >50 years.     

Integrated Expression of Circulating miR375 and miR371 to Identify Teratoma and Active Germ Cell Malignancy Components in Malignant Germ Cell Tumors

Active germ cell malignancies express high levels of specific circulating micro-RNAs (miRNAs), including miR-371a-3p (miR371), which is undetectable in teratoma. Teratoma markers are urgently needed for theselection of patients and treatments because of the risk of malignant transformation and growing teratoma syndrome. To assess the accuracy of plasma miR375 alone or in combination with miR371 in detecting teratoma, 100 germ cell tumor patients, divided into two cohorts, were enrolled in a prospective multi-institutional study. In the discovery cohort, patients with pure teratoma and with no/low risk of harboring teratoma were compared; the validation cohort included patients with confirmed teratoma, active germ cell malignancy, or complete response after chemotherapy. The area under the receiver operating characteristic curve values for miR375, miR371, and miR371-miR375 were, respectively, 0.93 (95% confidence interval [CI]: 0.87–0.99), 0.59 (95% CI: 0.44–0.73), and 0.95 (95% CI: 0.90–0.99) in the discovery cohort and 0.55 (95% CI: 0.36–0.74), 0.74 (95% CI: 0.58–0.91), and 0.77 (95% CI: 0.62–0.93) in the validation cohort. Our study demonstrated that the plasma miR371-miR375 integrated evaluation is highly accurate to detect teratoma.Patient summaryThe evaluation of two micro-RNAs (miR375-miR371) in the blood of patients with germ cell tumors is promising to predict teratoma. This test could be particularly relevant to the identification of teratoma in patients with postchemotherapy residual disease.     

Sacrococcygeal teratoma: A 33-year experience

During the years 1941 through 1973, 48 patients, 16 males and 32 females, with sacrococcygeal teratoma were seen at the Childrens Hospital of Los Angeles. Forty-four patients have been followed, three are lost to follow-up, and one patient died 2 wk after excision of teratoma.Of the 44 patients with follow-up, 26 had teratoma with mature tissues only, all these patients are living. Six patients had tumor containing mature and embryonic tissues. Of these, five are living and one died with metastases of malignant teratoma 1 yr after excision of the primary tumor. Of the remaining 12 patients, 11 have died during the first 4 yr of life due to malignant teratoma and only one is living without recurrence 15 yr after excision of teratoma containing frankly neoplastic tissues. Recurrence and/or metastasis of malignant sacrococcygeal teratoma was lethal in all instances.     

睾丸畸胎瘤核仁组成区嗜银蛋白定量研究

为进一步探讨睾丸良恶性畸胎瘤的鉴别方法,作者应用核仁组成区银染技术,对１０例睾丸正常组织、６例睾丸畸胎瘤、７例睾丸畸胎癌进行了研究。结果显示：正常睾丸及畸胎瘤的ＡｇＮＯＲｓ颗粒呈圆形、规则、边界清楚、大小均匀,位于胞核偏中或边缘部位;畸胎癌的颗粒呈圆形或卵圆形、欠规则、边界欠清、大小较不一致,偏中或散在分布于核内。ＡｇＮＯＲｓ计数：正常组为１．５６±０．１７,畸胎瘤为２．４０±０．２６,畸胎癌为５．２４±０．３６,各组间有显著差异（Ｐ＜０．０１）,ＡｇＮＯＲｓ计数在良恶性畸胎瘤间有分离。作者认为该技术对睾丸良恶性畸胎瘤的鉴别是有意义的。     

Adenocarcinomas arising from primary retroperitoneal teratoma in an adult female patient

Presented herein is a case of primary retroperitoneal teratoma with adenocarcinomatous transformation predominantly composed of signet ring cell carcinoma and intestinal-type adenocarcinoma in a 36-year-old woman. Retroperitoneum is an infrequent location for teratoma in adults and malignant transformation is exceptionally rare. Clinical manifestations, radiological studies, laboratory investigation, and pathological findings of this case are described. To the best of the authors' knowledge, 16 adult cases of primary retroperitoneal teratoma with malignant change have been reported in the literature. Herein is reported the first case of malignant teratoma with prominent component of signet ring cell carcinoma.     

Laparoscopic Abdomino-Paracoccygeal Resection of Anorectal Cystic Teratoma

Sacrococcygeal teratoma rarely presents in adulthood (reported incidence of 1:87 000). It is more common in females. Adult anorectal teratoma is a rare variant of sacrococcygeal teratoma. The majority of tumors spare the sacrococcygeal bone. The size and location of the tumor within the pelvis dictates whether it is approached surgically through a transabdominal, posterior, or a combined approach. We present in this article the case of a young woman with a large cystic anorectal teratoma treated successfully with a combined laparoscopic abdomino-paracoccygeal resection.     

Skull base reconstruction in cases of intracranial teratoma extending into the extracranial structures.

Recently experienced were two cases of postnatally diagnosed intracranial teratoma, one a mature teratoma and the other an immature teratoma, both extending into the extracranial structures. In each case, tumor resection was performed in which a barrier was created between the intracranial and extracranial spaces with a temporalis muscle flap. The technical aspect of skull base reconstruction is described, and previously reported cases of intracranial teratoma involving the skull base are reviewed.     

腹腔镜卵巢良性畸胎瘤手术40例体会

目的 :探讨应用腹腔镜行卵巢良性畸胎瘤手术的适应证与方式 ,预防和减少肿瘤破裂的发生。方法 :4 0例卵巢良性畸胎瘤患者行腹腔镜下囊肿剥除术。结果 :4 0例良性畸胎瘤手术均在腹腔镜下完成 ,无1例中转开腹 ,囊肿破裂 8例。平均手术时间 6 0min ,平均出血 80ml ,无术后出血、脏器损伤及化学性腹膜炎或肉芽肿的发生。结论 :应用腹腔镜行卵巢良性畸胎瘤的手术是完全可行的。