Extramammary Paget's disease arising in mature cystic teratoma of the ovary.

We report a case of extramammary Paget's disease in ovarian mature cystic teratoma. The patient was a 70-year-old Japanese woman who complained of lower abdominal pain. Examination showed elevation of carcinoembryonic antigen and CA 19-9. Ultrasonography and computer tomography revealed a cystic tumor of the left ovary. The gross appearance of the resected ovary was typical for mature cystic teratoma. Microscopic observation revealed a lesion of Paget's disease within the squamous epithelium. The tumor cells had intracytoplasmic mucin and positive immunoreactivity for carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin; but they were negative for S-100 protein and vimentin. On multiple and serial sections, underlying adenocarcinomas were not found either in the ovary or other primary sites. From these pathological findings, we concluded that the disease was an intraepithelial adenocarcinoma, possibly derived from multipotential cells in squamous epithelium of ovarian mature cystic teratoma. This is the first reported case, to our knowledge, of extramammary Paget's disease arising in mature cystic teratoma of the ovary.     

Uterine leiomyoma causing menometrorrhagia with a concomitant mature teratoma in a 15-year-old child: a case report and review of the literature

Uterine leiomyoma is the most common uterine tumor in adult females but is rare in the pediatric population with only 10 previous cases reported. We describe the unique case of a 15-year-old girl who presented with abdominal pain and menometrorrhagia and was found to have a uterine leiomyoma as well as a mature ovarian teratoma that required surgical resection. We review diagnostic imaging and optimal management for the 2 gynecologic masses in this teenage girl.     

Adult sacrococcygeal teratoma: a case report

We report a case of adult sacrococcygeal teratoma resected by an abdominosacral approach. A cystic mass 13 cm in diameter in the pelvic cavity and left hydronephrosis were detected incidentally by abdominal computed tomographic (CT) scan in a 55-year-old man. The pelvic tumor extending from the presacral area to the coccyx was resected via a combined abdominal and transsacral approach. The resected specimen weighed 700 g and the pathological diagnosis was mature teratoma. While the sacrococcygeal area is the most frequent site of teratoma in infants, it is a rare site in adults. This is to our knowledge, the 30th case report of adult sacrococcygeal teratoma in Japan. At one month after the operation the patient had no bowel dysfunction and no dysbasia, but he had mild bladder dysfunction requiring self-catheterization twice a day at twelve months. The patient had no evidence of disease at twenty months after the operation.     

Endoscopic surgery for mature teratoma of the ovary

Background: This study was undertaken to assess the value of laparoscopic surgery as treatment for benign mature teratomas of the ovary. Methods: A total of 70 patients treated exclusively with laparoscopic surgery for ovarian mature teratoma were studied. The tumors were either enucleated with preservation of the ovary or removed by salpingo-oophorectomy. Results: Ovary-preserving surgery was performed in 60 cases; salpingo-oophorectomy was accomplished in 10 cases. All tumors removed were histologically benign. No surgical complications were encountered throughout the series. In eight patients who underwent primary ovary-preserving surgery, a second-look laparoscopy was undertaken with virtually no pathological findings noted. Conclusions: Our findings confirm those of other authors—when performed by experienced surgeons, laparoscopic removal of ovarian mature teratomas is a safe and recommendable alternative to laparotomy. Preoperative diagnosis and intraoperative inspection of the tumor must be as thorough as possible.     

Renal cell carcinoma metastatic to the ovary: a case report]

A 47-year-old woman underwent left radical nephrectomy in 1995, and pathological diagnosis showed a primary renal cell carcinoma with clear cell subtype. Four years later on her routine checkup, abdominal computerized tomography revealed a 9-cm of predominantly solid and partially cystic tumor in the pelvic cavity. The patient was referred to Gynecologic Department and a total hysterectomy with bilateral salpingo-oophorectomy was subsequently performed under the diagnosis of a left ovarian tumor. A cut surface of the solid component of the tumor was macroscopically yellowish. Pathological examination revealed alveolar growth of tumor cells with abundant clear cytoplasm including fat components. In some areas of the tumor, there were patterns of tubular structures which were cystically dilated. The typical findings usually found in the primary ovarian clear cell adenocarcinoma were absent in the tumor, and the final pathological diagnosis was left ovarian metastasis of renal cell carcinoma. The ovarian metastasis of renal cell carcinoma is quite rare and to our knowledge only eleven cases were reported in the past 20 years. We report on a case and review the literature.     

Primary carcinoid tumor arising in a retroperitoneal mature teratoma in an adult

An extremely rare case of a primary carcinoid tumor arising in a mature retroperitoneal teratoma is reported. A 53-year-old woman was admitted for further examination of an incidental retroperitoneal mass with calcification. Computed tomography scans demonstrated a tumor with fat, soft tissue and bone densities on the left renal hilum. Surgical excision of the tumor was performed with a preoperative diagnosis of retroperitoneal teratoma. The pathological diagnosis was mature teratoma, including all three germ layers. A carcinoid tumor was evident among teratoid tissues and it was thought to be a teratoma with malignant transformation. The patient did not have a carcinoid syndrome and had an uneventful recovery. She has been followed for 31 months with no recurrence. Carcinoid tumors rarely occur in teratomas of the ovary and the testis and, to our knowledge, this is the first case of carcinoid arising in a retroperitoneal mature teratoma.     

A case of malignant teratoma developing from the septum pellucidum 7 years after removal of a mature teratoma in the pineal region

A report is presented on a case of teratoma with a malignant component arising from the septum pellucidum 7 years after total surgical removal of a mature teratoma in the pineal region. The patient was successfully treated with radiotherapy and total surgical resection of the second tumor. The case is characterized by the development of a second malignant tumor and by the long interval from complete resection of the primary tumor to the occurrence of the second tumor. Our case is not considered to be a recurrence of the mature teratoma of the pineal region but to be a multiple tumor of germ-cell tumor having multicentricity and a different temporal variety.     

41例卵巢成熟性囊性畸胎瘤腹腔镜手术治疗

目的　探讨卵巢成熟性囊性畸胎瘤腹腔镜手术的技巧和安全性。　方法　对 4 1例卵巢成熟性囊性畸胎瘤患者在腹腔镜下行肿物剔出术或附件切除术 ,采用自制标本袋盛装卵巢肿物 ,避免囊肿内容物污染盆腔。　结果　 4 1例中 4 0例在腹腔镜下完成手术 ,1例因手术困难而中转开腹手术。术后随访 4月～ 6年 ,均未见复发。　结论　腹腔镜手术治疗卵巢成熟性囊性畸胎瘤是一种安全、有效的方法 ,具有出血少、损伤小、恢复快、无远期并发症等优点。     

Multilocated mature teratoma: a case report and review of the literature

Summary Mature teratomas are rare inclusion tumors, which have benign behaviors. Different locations of the tumor were reported in the literature. However, multiple tumor locations are extremely rare. An eight year old patient presented with an intraventricularly mature teratoma. During the six year follow up-period, two separately located masses were observed in the chest area and in the right iliac region, and both tumors were histologically diagnosed as mature teratomas. In this study, this unique case of mature teratoma presented with multiple locations in three separate body compartments. Furthermore, intraventricular dissemination with sellar and parasellar extension was observed in the third ventricle.     

Mucinous cystadenoma arising 3 years after ovarian-sparing surgery for mature teratoma in a child

We report the case of a 15-year-old girl diagnosed with mucinous cystadenoma 3 years after ovarian-sparing surgery for a mature teratoma located in the same ovary. Ovarian teratoma is the most common ovarian neoplasm in children, whereas mucinous cystadenoma is extremely rare during childhood.     

Primary carcinoid tumor in a retroperitoneal mature teratoma: report of a case

Primary retroperitoneal teratoma in an adult is rare, as is the occurrence of a malignant tumor within a mature teratoma, known as “malignant transformation”. A 24-year-old woman was admitted to our hospital for investigation of an abdominal mass. Computed tomography and magnetic resonance imaging revealed a multilocular mass in the right upper abdomen. The tumor consisted of fat, soft tissue, and bone, with a slightly enhanced solid component. The tumor was diagnosed preoperatively as a retroperitoneal mature teratoma with an immature component, and excised. Histologically, it was composed mainly of mature fat, soft tissue, and bone, accompanied by a solid component of prostate-like tissue. In addition, a latent carcinoid tumor was recognized in the middle of the tumor. The tumor was finally diagnosed as a primary carcinoid tumor within the retroperitoneal mature teratoma. The patient has been followed-up for 24 months since her operation without any evidence of recurrence. We report this case to highlight the possibility of malignant transformation in adult retroperitoneal teratoma, even when the preoperative diagnosis is benign mature teratoma.     

Mature cystic teratoma of the ovary with differentiated and secretive gastric mucosa

Large teratomas with gastric mucosa are rare. We report a case of mature cystic teratoma in an ovary of a young woman. Tumor growth was large and rapid enough to displace abdominal organs. The cyst was aspirated (removing 101 of mucinoid liquid) and excised by medial hypogastric laparotomy. The histological examination revealed differentiated gastric fundal mucosa with no other tissue involvement.     

Mature metastasis of a mature testicular teratoma as differential diagnosis of a renal/adrenal tumor

Summary We report on the eighth known case of a mature metastasis of a mature teratoma of the testis. In a 19-year-old patient who had undergone a pyeloplasty of the left renal pelvis 6 years before, a mature metastasis of an occult mature teratoma of the testis was found at the former operation site. This case – in addition to those published previously – emphasizes the fact that a mature teratoma of the testis has to be regarded as a malignant tumor in adults. It is supposed that the mature teratoma originates, as other testicular tumors, from carcinoma-in-situ-cells, which are responsible for metastasize and are not detected after lysis.      

Metachronous benign ovarian tumors are not uncommon in children

Purpose

To evaluate the risk for metachronous ovarian tumor in pediatric patients with mature ovarian teratoma.

Methods

During 1981–2011, 22 children underwent oophorectomy for mature teratoma at the median age of 11.4 (range 1.5–15.3) years. The patients were followed-up in median 4.4 (range 0.5–25.5) years.

Results

None of the patients had synchronous bilateral tumor at the time of primary operation, but during follow-up five patients (23%) got metachronous contralateral ovarian tumor. The contralateral tumor was observed in median 3.6 (range 1–8.8) years after the primary operation. According to Kaplan–Meier analysis the risk for contralateral tumor was 14% ± 8% (SE) within five years and 66% ± 26% (SE) within 10 years. In this series, the contralateral tumor was operated by ovary preserving surgery. Three of the metachronous tumors were mature teratomas and two were seromucinous infantile cystadenomas. One patient had a second teratoma recurrence 14 years after the first recurrence.

Conclusions

More than one fifth of the children with ovarian mature teratoma get metachronous benign tumor to the contralateral ovary. Therefore a yearly ultrasound follow-up is needed for these patients up to potential pregnancy to enable early diagnosis, ovary preserving surgery and maintenance of fertility in the case of metachronous tumor.     

Embryonal rhabdomyosarcoma arising in a mature teratoma of the testis: a case report

We report a case of embryonal rhabdomyosarcoma present in the primary excision of an intratesticular purely mature teratoma. Testicular mature and immature teratomas are usually associated with other germ cell tumors. Nongerminal malignancies that tend to occur in gonadal and extragonadal teratomas are often epithelial. Sarcomas of all types are less frequent but embryonal rhabdomyosarcomas are encountered predominantly. This sarcomatous element can be present in the primary excision or it can appear after chemotherapy in the metastases. Usually, prognosis is dependent on the degree of aggressiveness of the sarcomatous component. A review of the literature reveals that it is unusual for an embryonal rhabdomyosarcoma to develop on purely mature teratoma. We report a case in the testicle. During followup metastases arise rapidly from the purely embryonal rhabdomyosarcomatous component. The accurate diagnosis of this unusual sarcomatous component is of paramount importance because of the implications for therapy.     

Surgical management of growing teratoma syndrome: the M. D. Anderson cancer center experience

PURPOSE: We defined the growth rate and reviewed our experience in the surgical management of growing teratoma syndrome in patients with nonseminomatous germ cell tumors. MATERIALS AND METHODS: Nine patients were clinically diagnosed with growing teratoma syndrome at our center from 1980 to 2003. The defining criteria of growing teratoma syndrome were growing metastatic mass in the retroperitoneum or other site consisting entirely of mature teratoma detected on serial abdominal/pelvic imaging during chemotherapy, and a significant decrease in or normalization of tumor markers. RESULTS: Precise serial abdominal/pelvic radiological measurements of the retroperitoneal masses were available for 7 of 9 patients. The median growth rate of growing teratoma syndrome measured as the increase in diameter during chemotherapy was 0.7 cm per month. The median growth rate measured as the increase in tumor volume was 12.9 cc per month. Median time from the start of chemotherapy to retroperitoneal lymph node dissection was 5.4 months (range 2.7 to 21.6). Pathological evaluation of the retroperitoneal lymph node dissection specimen revealed teratoma with no viable tumor in all 9 cases. There were 2 intraoperative complications (1 aortic and 1 ureteral injury) and 4 postoperative complications (2 ileus, 1 acute pancreatitis and 1 chylous ascites). At a median followup of 2 years 7 patients were alive without disease, 1 died of postoperative sepsis and 1 died of an unknown cause. CONCLUSIONS: The growth rate of growing teratoma syndrome can vary significantly, which must be taken into account when evaluating cases. Retroperitoneal lymph node dissection is an effective treatment for growing teratoma syndrome, providing excellent local control and a low risk of progression.     

Renal teratoma with duplication of cecum and appendix

Renal teratoma is a very rare condition. We report a case of mature renal teratoma in a 1-month-old male infant. The patient was admitted with a right-sided abdominal mass. The mass was found to arise from the right kidney. The tumor was removed successfully, and the patient had an uneventful recovery. The tumor mass also had an appendix and cecum within the tumor, along with a normal appendix and cecum with a separate blood supply.     

Malignant melanoma involving the ovary: a clinicopathologic and immunohistochemical study of 23 cases

Ovarian malignant melanoma (MM), primary or metastatic, is an extremely rare tumor and in the absence of a previous diagnosis can represent a diagnostic challenge. We present the clinicopathologic and immunohistochemical features of 23 cases seen in our institution over a period of 40 years (1962-2001). The patients' age ranged from 14 to 53 years (mean 35.7 years). Ethnicity was known in 19 patients: 14 white, 4 Hispanic, and 1 black. A previous history of MM was definitively obtained in 14 patients; in these cases, the interval between the primary MM and the ovarian metastasis ranged from 15 to 228 months (mean 77.7 months). The tumor was unilateral in 19 and bilateral in 4 cases. The tumor size ranged from 4.5 to 23 cm (average 10 cm); the melanoma arising in a cystic teratoma was 0.2 mm in thickness. The tumor was grossly pigmented in 8 cases (35%). The architectural pattern was nodular (8 cases), diffuse (6 cases), nodular and diffuse (5 cases), nested (3 cases), and lentiginous arising in a teratoma (1 case). Follicle-like spaces were seen in 8 cases, pseudo-glandular areas in 1 case, pseudo-myxoid areas in 1 case, and cords in 1 case. The tumor cell type was epithelioid in 19 cases, spindled in 2 cases, mixed epithelioid and spindled in 1 case, and small cell in 1 case. Nucleoli were prominent in 18 cases, and nuclear inclusions were present but rare in the majority of cases. Nuclear grooves were seen in 3 cases. Necrosis was extensive in 8 cases, focal in 10 cases, and was absent in 5 cases. In 8 cases, initial diagnoses included sex cord stromal tumor, germ cell tumor, sarcoma, or undifferentiated carcinoma. S-100 was positive in 18 of 19 cases, HMB-45 in 17 of 20 cases, MART-1 in 13 of 15 cases, tyrosinase in 10 of 15 cases, and Mitf in 8 of 14 cases. Inhibin was positive in 3 of 14 cases. Calretinin was focally positive in 1 of 12 cases. Treatment performed in 18 of the cases are as follows: oophorectomy with/without chemotherapy (10); total abdominal hysterectomy with bilateral salpingo-oophorectomy with/without chemotherapy (6); vaginal hysterectomy, bilateral salpingo-oophorectomy, and chemotherapy (1); and total abdominal hysterectomy with salpingo-oophorectomy (1). Follow-up ranging from 2 to 96 months was available in 18 patients. All but one had metastases in other organs, most often in the lungs. Thirteen patients died of disease (range 2-76 months), 3 are alive with disease (6-18 months), and 2 have no evidence of disease at 24 and 96 months; one was the patient with melanoma arising within a teratoma. In conclusion, MM involving the ovary is a rare disease, predominantly seen in women of reproductive age, and is associated with a poor prognosis. The tumor is most often metastatic from another site and is unilateral in most cases. Nodular or diffuse pattern and epithelioid cell type are most frequently seen, and the tumor can be mistaken for germ cell and sex cord stromal tumors. S-100 is the most sensitive marker. MART-1 was positive in the few cases that were negative with HMB-45. Inhibin can be focally positive in some cases.     

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.