The current case report presents an account of a unique surgical procedure performed to remove an extremely rare occurrence of a transsacral abdominal mass from a 24-year-old female. The patient presented with subtle sacrococcygeal pain for two months and a presacral abdominal mass derived from the right ovary. The mass was misdiagnosed as a presacral tumor based on the results of magnetic resonance imaging (MRI) performed prior to the surgery. The patient also exhibited the symptoms commonly caused by a presacral mass, however, during the surgery, the mass was not initially located under the sacrum. An ultrasound examination and an analysis of an intraoperative frozen section indicated that the mass was a mature cystic teratoma (MCT) of the ovary, located in the peritoneal cavity between the rectum and uterus. The mass was successfully resected and removed from the affected ovary through the abdominal cavity via the sacral region. A pathological examination of the tumor section confirmed a diagnosis of a MCT of the ovary.
mature cystic teratoma; ovary; presacral tumor; transsacral approach
Intracranial nongerminomatous germ cell tumors (NGGCTs) in unusual locations are extremely rare. Here, we report a case of a yolk sac tumor in the frontal lobe in a middle-aged patient. A 42-year-old man was admitted to our hospital for headache and nausea. Magnetic resonance imaging (MRI) showed an enhanced mass lesion with a marked cyst component. The serum alpha-fetoprotein (αFP) level was extremely high. Histological examination of specimens after subtotal removal revealed a primary mixed germ cell tumor with extensive yolk sac tumor elements, often referred to as an intracranial “yolk sac tumor.” The preoperative diagnosis of NGGCTs in unusual age and locations is extremely difficult. Clinicians should consider the possibility of NGGCTs, including yolk sac tumors, when intracranial tumors with unusual MRI findings are encountered.
Yolk sac tumor (endodermal sinus tumor) is a rare malignant germ cell tumor arising in the testis or ovary. Extragonadal yolk sac tumor is even rarer and has only been described in case reports. Due to the rarity of the tumors, the appropriately optimal treatment remains unclear. We report a case of yolk sac tumor in the seminal vesicle.
A 38-year-old Asian male presented with gross hematuria and hemospermia. Transrectal ultrasound scan showed a solid mass in the left seminal vesicle and the scrotal sonography showed no abnormalities. Bilateral seminal vesicles were resected, and histopathological examination showed a typical pattern of yolk sac tumor (YST). The patient responded poorly to comprehensive treatment of radiotherapy, chemotherapy and surgeries, developed systemic multiple metastases, and died of cachexia one and half years after diagnosis.
Yolk sac tumor; Seminal vesicle; Extragonadal
Yolk sac tumor (YST) is a rare malignant tumor originating from germ cells. YST normally originates from the gonads, rarely occurring in extragonadal sites. We report a 35-year-old man with YST arising in the omentum, which is the first reported case of a primary YST of the omentum in an adult male. The patient presented to the community hospital with abdominal distension. A CT scan showed thickening of the omentum with ascites. The patient underwent open biopsy of the omental mass. The Pathology Department of the hospital could not make a definitive diagnosis at that time, and the tumor was considered a cancer of unknown primary (CUP) origin with features of primary colorectal cancer based on the immunohistochemistry (IHC) findings of the biopsy specimen (CK7–/CK20+ and CDX-2+). He was then referred to our hospital. We found that serum α-fetoprotein was abnormally elevated to 7,144 ng/ml (normal <10.0), and reevaluation of the biopsy specimen revealed microcystic or reticular patterns of tumor cells with Schiller-Duval bodies typical of YST. The present case suggests that IHC is a very useful diagnostic tool for subtyping CUP but should be interpreted in the context of clinical and morphological findings.
Germ cell tumor; Yolk sac tumor; Omentum; Immunohistochemistry
Endodermal sinus tumor (or Yolk Sac tumor) of the vagina is a rare malignant germcell tumor which is seen exclusively in children younger than 3 years of age. We report two cases of endodermal sinus tumor of the vagina. In both cases no radiological investigation was done and serum alpha-fetoprotein was elevated. The histopathological examination of both the tumor masses revealed vaginal endodermal sinus tumor. Periodic-acid-Schiff stain with diastase showed diastase resistant hyaline globules. These findings confirmed the diagnosis of endodermal sinus tumor in both cases. Vaginal endodermal sinus tumor is both locally aggressive and capable of metastasis. The serum alpha-fetoprotein level is a useful marker for diagnosis and monitoring the recurrence of vaginal endodermal sinus tumor in infants. Early detection and therapy is important because of its aggressive nature and good response to chemotherapy.
yolk sac tumor; endodermal sinus tumor; vagina; germ cell tumor; alpha-fetoprotein
Extragonadal yolk sac tumor (YST) is a relatively rare entity. We describe here the case of an extragonadal YST that occurred in the pancreas with hepatic metastasis in an adult woman. The contrast enhanced CT images of the abdomen revealed a heterogeneous, solitary mass occupying the pancreatic neck and body with slightly inhomogeneous contrast enhancement. Two low-density lesions in the liver were also displayed on the CT images. The patient underwent surgery and the diagnosis of YST was pathologically verified.
Yolk sac tumor; Pancreas; Computed tomography (CT); Diagnosis
Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4,000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000–1:200,000 live births. This accounts for 2–9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. We present a case of a female infant with a combination of nasopharyngeal teratoma and a cleft palate with successful multidisciplinary management via a staged surgical approach. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral extirpation and cleft palate repair at a later stage, was performed. The results of the histopathological examination confirmed diagnosis of a congenital teratoma.
Epignathus; Nasopharyngeal teratoma; Cleft palate
Tumors of the sacrum and related neurological and pelvic structures are rare pathologic conditions.
Sacral tumors are a diagnostic challenge because patients often present with nonspecific complaints such as low back pain, sacrococcygeal joint pain, referred lag and buttock pain. Because of diagnostic and radiographic challenges, the detection of sacral mass can be delayed which complicates any prospect surgical intervention as tumors can be quite large at the time of treatment. Furthermore, the complex local anatomy of the sacrum and presence of numerous other structures make the resection procedure even more sophisticated and necessitate adoption of a multidisciplinary approach towards the treatment. The present study discuses the diagnosis, treatment and complication of 4 cases with large sacral mass.
Resection, Sacral, Tumor
Objective: Sacrectomy creates a large, complex tissue defect that presents a reconstructive challenge for plastic surgeons. Several myocutaneous flaps have been described for reconstruction following sacral tumor extirpation; however, current publications focus on the reconstructive options applicable to adults. We present a method of reconstruction following sacral tumor extirpation in a pediatric patient. Methods: The patient was 22 months old and in need of complex closure following low sacral amputation (S3-S4 osteotomy) and en bloc resection of a yolk sac tumor. Following tumor extirpation, the patient was left with a complex defect including extensive dead space, multiple exposed nerve roots, projection of the rectum into the wound, and inadequate soft tissue for primary closure. Results: Reconstruction with human acellular dermal matrix to address the risk of posterior rectal herniation and bilateral gluteal V to Y advancement flaps for obliteration of the dead space allowed for durable closure of the surgical defect. Conclusions: This represents the first case report documenting sacral resection and reconstruction with bilateral V to Y gluteal advancement flaps in a pediatric patient.
To present clinical and histological features of prepubertal testicular tumors through the analysis of the long-term experiences of a single surgeon.
Materials and Methods
The charts of 48 children treated for testicular tumors from 1986 to 2010 were retrospectively reviewed. All patients underwent radical orchiectomy. The patients' ages, clinical presentations, histopathological findings, kinetics of tumor markers, and outcomes were recorded.
The patients' median age at the initial diagnosis was 19.5 months (range, 3 to 84 months). All patients presented with either a palpable mass (76%) or scrotal size discrepancy (24%). Compared with a palpable mass, scrotal size discrepancy led to delay in diagnosis by 5 months. Regarding histology, yolk sac tumors and teratomas accounted for 53% and 36% of the tumors, respectively. The mean preoperative alpha-fetoprotein (AFP) level was significantly higher in patients with yolk sac tumors than in those with teratomas (4,600 ng/mL vs. 6.3 ng/mL), and only one patient with a teratoma had a preoperative AFP value higher than 20 ng/mL. Following radical orchiectomy, 72%, 8%, and 16% of patients with a yolk sac tumor showed normalization, persistent elevation, and relapse after transient lowering of AFP, respectively. Preoperative AFP was greater in patients with non-normalization than in those with normalization. Five of six patients with non-normalization showed evidence of either vascular invasion or endolymphatic tumor emboli.
We found a higher number of yolk sac tumors than teratomas in patients with prepubertal testicular tumors. AFP was the most useful marker in the diagnosis and follow-up of childhood yolk sac tumors. Relapsed yolk sac tumors often showed pathological evidence of aggressiveness.
Adolescent; Child, Preschool; Race relations; Testicular neoplasms
Transverse testicular ectopia (TTE) is a rare anomaly in which both testes descend through a single inguinal canal. We report a case of yolk sac tumor in the ectopic testis of a patient with TTE. A 24-year-old man presented to our hospital with a left inguinal-mass, right cryptorchidism and elevated alpha-fetoprotein (AFP). A left herniotomy 3 years earlier demonstrated both testes in the left scrotum, one above another positionally. Four months ago, a left scrotal mass appeared and radical orchiectomy of both testes revealed testicular yolk sac tumor of the ectopic testis. An enlarging left inguinal-mass appeared 2 months ago and he was referred to our hospital. Laboratory data showed an elevation of AFP (245.5 ng/ml) and a 46 XY karyotype. He underwent bilateral retroperitoneal lymph node dissection and simultaneous left inguinal mass dissection. Histopathologic examination revealed a diagnosis of recurrent yolk sac tumor in the left inguinal mass. The retroperitoneal lymph node was not enlarged and, on histopathology, was not involved. The patient has now been followed up for 8 months without evidence of biochemical or radiological recurrence.
Since gonadal yolk-sac tumour in pure form or as a component of mixed germ cell tumour is in the majority of patients highly malignant, its histological recognition is of great prognostic importance. Yolk-sac tumour may assume various different histological guises, which have hitherto caused considerable terminological confusion; the present paper is aimed at correlating these morphological diversities with biochemical features which are consistent with yolk-sac differentiation. Using an enzyme-bridge immunoperoxidase technique, a series of 16 gonadal germ cell tumours with a yolk-sac component were screened for the presence of alpha-fetoprotein, alpha-1-antitrypsin, and transferrin. These proteins, normally produced by human yolk sac, were demonstrable in all the morphological patterns of yolk-sac tumour we have previously described. Six malignant non-germ cell tumours were submitted to the same investigations, and no evidence of the three protein markers was found in five; one tumour, however, an oat cell carcinoma of the bronchus, stained positively for transferrin.
Germ cell tumor (GCT) of the liver is extremely rare. Here, we describe a case of hepatic mixed GCT with significant sarcomatous components and elevated serum α-fetoprotein (AFP) in a 34-year-old man. Histopathologically, the tumor was composed of two GCTs components: yolk sac tumor and immature teratoma. The predominant components of immature teratoma consisted of several types of tissue that represented different germinal layers (endoderm, mesoderm and ectoderm) and showed varying degrees of differentiation with significant sarcomatous components. The yolk sac component showed positivity for AFP and cytokeratin (AE1/AE3). The immature teratoma components showed positivity for varying differentiation markers. Interphase cytogenetic analysis revealed that the yolk sac tumor and immature teratoma were positive for i(12p) and 12p over-representation. In particular, the rhabdomyoblastic components also showed typical i(12p) and 12p overrepresentation. This suggested that sarcomatous components may be associated with dedifferentiation or malignant transformation of certain mesenchymal components within teratoma.
Germ cell tumor; Teratoma; Sarcoma; Liver neoplasms
Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma (NPT) associated with a wide cleft palate.
A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6×4×3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma (hairy polyp). The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development.
Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction.
Teratoma; Nasopharyngeal Tumor; Cleft Palate; Neonate; Airway Obstruction
Primary yolk sac tumour of the liver is exceedingly rare. A 28 year old woman presented with a cystic liver mass and a markedly raised serum alpha-fetoprotein concentration. She underwent a partial hepatectomy for a suspected hepatocellular carcinoma but histological examination of the tumour revealed the classical morphological and immunohistochemical features of a yolk sac tumour. There was no evidence of an extrahepatic primary source. Review of this case, together with the six previously reported adult cases of primary yolk sac tumours of the liver, revealed several features of the tumour that may aid differentiation from hepatocellular carcinoma, with potential therapeutic implications.
Gastric teratomas are very rare tumours in children. They usually present with a palpable mass in the upper abdomen. We report a case of gastric teratoma in one and half month old male infant who presented with a palpable mass in abdomen, extending from epigastrium to the pelvis. Ultrasound of abdomen revealed a huge mass with solid and cystic components. CT scan delineated calcifications in the mass. The preoperative diagnosis was a teratoma but not specifically gastric one. The mass was excised completely with seromuscular layer of the stomach wall. The histopathology confirmed it to be grade-3 immature gastric teratoma. The rarity of the origin of teratoma in addition to its immature variety prompted us to report the case.
Gastric teratoma; Immature teratoma; Infant
To report on a female patient diagnosed with Currarino’s triad in adulthood.
This case presents an adult patient with a medical history of a congenital anal atresia, a partial sacral agenesis, and a surgically treated ectopic anus. After a coincidentally observed presacral mass by MRI, due to unexplained constipation later in adulthood, Currarino’s triad was suspected in this patient. This triad consists of anorectal malformation(s), sacrococcygeal defects and a presacral mass of various origin. Further investigation confirmed the mass to be a meningocele, and showed a tethered cord and a syrinx.
In (young) patients with anorectal malformations, although having no other symptoms, further examination might be required to exclude Currarino’s triad. Importance of early diagnosis and multidisciplinary assessment is recommended to establish adequate treatment if needed.
Currarino; Anorectal malformation; Sacrococcygeal agenesis; Presacral mass; Meningocele
Yolk sac tumour is the most common germ cell tumour in infants and children, with a majority of them arising in the gonads. Rare cases of extra – gonadal germ cell tumours have been described in the literature. We are presenting here, a case of a yolk sac tumour of the cryptorchid testis in a 2 year old child, who initially presented with a mass in the left lobe of the liver, with huge ascites and which posed diagnostic difficulties. The mass was diagnosed as hepatoblastoma on Computed Tomography (CT). Subsequently, CT guided Fine Needle Aspiration Cytology (FNAC) of the liver mass showed the features of a yolk sac tumour. The raised serum Alfa Foetoprotein (AFP) levels corroborated with the cytological diagnosis.
Alfa foetoprotein; Fine needle aspiration cytology; Cryptorchidism; Yolk sac tumour
This study reviews a case of a large cystic anorectal teratoma successfully treated with a combined laparoscopic abdomino-paracoccygeal resection.
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.
Sacrococcygeal teratoma; Anorectal teratoma; Laparoscopic excision; Abdomino-paracoccygeal
We describe here a case of pure gastric yolk sac tumor (YST). A 62-year-old patient underwent gastrectomy with D2 dissection. The histological report confirmed the diagnosis of YST and that two of the 14 regional lymph nodes removed were metastatic. Three courses of PEB regimen chemotherapy were delivered subsequently. Three months later the patient experienced dysphagia from stenosis of the anastomosis and a computerized tomography scan showed tumor recurrence with peritoneal nodules; the patient died one year after surgery. The origin of gastric YST is unclear but involvement of migrating germ cells during embryonic development or multipotential neoplastic protoepithelial cells of the gastric mucosa have been suggested. Generally the prognosis of gastric YST is poor and the standard therapeutic approach beyond surgery is still uncertain.
gastric cancer; yolk sac tumor; chemotherapy; biology.
Teratoma is a tumor that forms triploblastic tissues and the common sites of occurrence are sacrococcygeal lesions and the ovaries. The majority of cases are curable with surgical resection and the prognosis depends on the extent and histological scoring of the tumor. In the present study, we report a case of sacrococcygeal teratoma of a newborn showing features of a retina-like structure. A 29-year-old woman gave birth prematurely to an infant girl with sacrococcygeal teratoma. Surgical resection was performed 10 days after delivery. The tumor contained immature components as well as a retina-like structure. Several investigations, including immunohistochemical analysis, confirmed the similarities between the normal mouse retina and the retina-like structure of the tumor. The vascular arrangement and polarity surrounding the retina-like structure are unique and this is thought to be significant in the induction of structural differentiation. Our findings may provide insights into the matter of teratogenic activity in stem cell therapies for clinical applications.
sacrococcygeal; teratoma; retina
Sacrococcygeal location of myxopapillary ependymoma (MPE) is uncommon. Local recurrence and metastases are on record inspite of its benign characteristics. We report a rare case of sacrococcygeal MPE in an 11-month-old female child who showed typical myxopapillary ependymal histology along with anaplastic ependymal component. Ki-67 labeling index in the myxopapillary component was 4-5% and in the anaplastic component was 70%. Six weeks after gross total resection of the tumor, the child presented with local recurrence and metastasis in the right inguinal lymph nodes and was treated with chemotherapy. The present case of sacrococcygeal MPE with anaplastic ependymoma component is the second case on record in the medical literature, and the first case without any syndromic features. Metastasis in this case can be explained because of the anaplastic component, with mitotic count of 5-6/high power field and high Ki-67 labeling index.
Anaplastic ependymoma; Ki-67; myxopapillary ependymoma; sacrococcyx
A nongerminomatous germ cell tumor occurring in the brain parenchyma is extremely rare. A 2-year-old boy presented with symptoms of abnormal movements in the hand and mouth. MRI scanning revealed a lesion occupying the left temporoparietal region. Craniotomy was performed and the tumor was removed by en bloc resection. Histological examination revealed that the tumor was arranged in a reticular pattern, and Schiller-Duval bodies were evident at the center of the tumor. Immunohistochemical study showed that the tumor cells were positive for alpha-fetoprotein and vimentin, but negative for glial fibrillary acidic protein. The histological diagnosis was pure yolk sac tumor).
Paratesticular germ cell tumors are extremely rare. A 12-month-old boy with yolk sac tumor involving only the paratesticular tissue is reported. Pre-operatively raised alpha fetoprotein levels fell to normal levels after high inguinal orchiectomy. This appears to be the youngest and only the 3rd case reported in the English literature.
Alpha fetoprotein; paratesticular tumor; primary extragonadal germ cell tumor; yolk sac tumor
A xenograft line, HX 53, has been established in immune-suppressed mice from a specimen of a lymph node metastasis in a patient with a histological diagnosis of seminoma but with markedly raised circulating levels of alpha-fetoprotein. Histological, immunocytochemical, and ultrastructural studies of this xenograft line have suggested that a solid variant of yolk sac carcinoma may exist, which morphologically resembles seminoma, or that a continuum of differentiation exists between seminoma and yolk sac carcinoma.