Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma.
Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy.
Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery.
This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms.
The authors report a rare case of mesenteric monodermal teratoma that mimicked an ovarian tumor and was successfully managed with hand-assisted laparoscopic intervention.
Mature cystic teratomas are benign neoplasms of germ cell tumors that occur most frequently in gonadal sites. The tumors usually contain 2 or 3 well-differentiated elements of endodermal, ectodermal, and mesodermal origin. Although relatively uncommon, teratomas can be composed of mature tissue originating from only 1 germ cell layer. This is known as a monodermal teratoma.
Extragonadal teratomas, especially mesenteric teratomas, are extremely rare. Currently, only 21 cases of mesenteric teratoma have been described in the English literature. Mesenteric teratomas are rarely diagnosed preoperatively because pathological examination is necessary to make a definitive diagnosis.
We herein report a rare case of mesenteric monodermal teratoma and review the literature. To the best of our knowledge, this is the first case of mesenteric teratoma treated with hand-assisted laparoscopic surgery.
Hand-assisted laparoscopic surgery; Laparoscopy; Mature cystic teratoma; Mesenteric cyst; Ovarian cyst
We report a case in which an immature teratoma developed following three previous resections for mature cystic teratomas. The patient was a 26-year-old nulliparous woman with a regular menstrual cycle. Twelve years earlier, she had consulted a pediatrician for complaints of lower abdominal pain. Bilateral cystic teratomas were suspected and she underwent a left salpingo-oophorectomy and a right cystectomy laparoscopically, and bilateral mature cystic teratomas were diagnosed histologically. She underwent a right cystectomy twice afterwards and mature cystic teratomas were diagnosed. Three years after the third surgery, a regular checkup performed annually for ovarian cyst recurrence revealed a 9.3 cm ovarian cyst by ultrasonography without marker elevation or complaint of symptoms. Magnetic resonance imaging (MRI) showed a 10 cm multilocular cyst, including a part with heterogeneous medium and high-signal intensity on T2-weighted images, which revealed enhancement on dynamic contrast-enhanced MRI unlike the previous images. Ovarian tumors, including immature teratomas and malignancy, were considered. She had a strong wish to undergo laparoscopic surgery. She was diagnosed with an immature teratoma, grade 1 of the right ovary. Although the frequency of recurrence of immature teratomas after resection of mature cystic teratomas is very low, regular checkups are necessary because there may be no associated symptoms.
While mature cystic teratoma of the ovary is the most common ovarian tumor in young women, immature teratoma is a very rare tumor, representing only 1% of all ovarian cancers. In the three cases presented here, young women who were suspected to have mature cystic teratoma, based on CT scan and MRI, were ultimately diagnosed with immature teratoma Ic (b) G1 after laparoscopic operation. They underwent salpingo-oophorectomy of the affected side only and have shown no sign of recurrence during follow-up. We sometimes encounter patients with immature teratoma who have no findings pointing to malignancy on CT or MRI. Generally, if the components of immature nerve cells that represent immature teratoma are very few, it is difficult to diagnose the entity as immature teratoma with imaging evaluations such as CT or MRI. In many hospitals, laparoscopic surgery is selected for patients with ovarian mature teratoma. Therefore, it is essential to attempt as much as possible not to disseminate the fluid content of the tumor into the intra-abdominal space during laparoscopic operation, because in rare cases the tumor turns out not to be benign mature teratoma.
mature teratoma; immature teratoma; laparoscopic surgery
Teratoma refers to a neoplasm that recapitulates all three germ layers. Teratomas may be histologically mature and oncologically benign. Teratomas may also be histologically immature while being oncologically benign, or they may harbor malignant components and have the potential to exhibit an aggressive biological behavior. Teratomas of the head and neck are extremely rare and usually present in the neonatal period. As a general rule, pediatric teratomas of the head and neck tend to be oncologically benign, whereas adult teratomas tend to be histologically and oncologically malignant. Most of these teratomas are found in the cervical region and nasopharynx. Calcification within the mass is often evident.
A 27-year-old Caucasian man complaining of a nasal obstruction was admitted to our clinic in January 2006. A transnasal endoscopic examination revealed a mass arising from the nasal septum which was completely removed using an endoscopic approach. Histologically, it was determined to be a benign teratoma.
Herein, we present a rare case, along with a review of the related literature, in order to emphasize that a benign teratoma of the nasal septum should not be ignored.
One hundred and forty six patients with advanced germ cell testicular tumours (38 seminomas and 108 malignant teratomas) treated by combination chemotherapy were studied over 10 years. Most of the improvement seen was in patients with malignant teratoma undifferentiated. In the most recently treated patients (1984-1987) 75% of drug resistant cases were malignant teratoma intermediate compared with 26% in the series treated between 1978-1983. The microscopic features of 52 primary testicular tumours were compared with features seen in excised retroperitoneal lymph nodes after completion of chemotherapy. Primary malignant teratoma intermediate had a higher incidence of viable malignancy in the excised tissue than malignant teratoma undifferentiated. Mature teratoma or fibronecrotic tissue within resected tissue was associated with a good prognosis. If resection was complete patients with drug resistant malignant teratoma intermediate had a more favourable prognosis than drug resistant malignant teratoma undifferentiated. It is advised that retroperitoneal node dissection should be considered in the management of patients with advanced germ cell testicular tumours, and that as complete a resection as possible be attempted to avoid the danger of missing residual disease.
Cystic mature teratomas of the spinal cord are rare lesions. Teratomas account for up to 0.1% of all spinal cord tumors. Teratomas include tissues that originate from the three germ layers. Several congenital disorders may accompany the teratoma. Teratomas are classified as mature, immature or malignant type according to their histological characteristics. Thoracic spinal teratomas are uncommon in the pediatric age group. More than half of the patients are adults. We present herein a five-year-old male patient who was referred to our clinic with cystic mature teratoma at the T12 level.
Mature teratoma; spinal tumor; thoracic region; treatment
Several serine protease enzymes are known to be involved in both normal desquamation and the inflammatory processes of the skin. Alteration in the activity of these proteases should also affect corneocyte maturity and size as well as stratum corneum thickness. The aim of the present work was to characterise the baseline changes in corneocyte size, corneocyte maturity, selected protease activity (specifically, Kallikreins-5 and 7, tryptase), protein content and trans-epidermal water loss (TEWL) as a function of anatomic site. The anatomic sites investigated were: cheek, abdomen, wrist and mid-ventral forearm. TEWL values were highest for the cheek (p < 0.05). The TEWL values were also significantly higher (p < 0.05) for cheek and wrist compared with other sites. Protein content was significantly lower for wrist (p < 0.05) compared with other sites. Corneocyte maturity and surface area were significantly (p < 0.05) lower for cheek and wrist compared with other sites. An excellent correlation (r2 = 0.99) was obtained for maturity and surface area measurements. Kallikrein-5 and tryptase activity were significantly higher for the cheek compared with other sites but Kallikrein-7 values were uniform across sites. The findings have significant implications for skin permeability to drugs and other substances such as environmental toxins depending on the anatomic site of delivery or exposure.
kallikrein; maturity; protease; skin; transepidermal water loss
Teratoma is a germ-cell tumor that commonly affects the gonads. Its components originate in the ectoderm, endoderm, and mesoderm. Extragonadal occurrence is rare. Teratomas confined to the adrenal gland are exceptional; only 3 cases have been reported in the English-language literature. We report 2 cases of mature teratomas of the adrenal gland that were laparoscopically excised.
Two patients (ages 8 and 61 years) were diagnosed with adrenal teratoma at our institution. Radiological examination showed a solid 8-cm adrenal lesion in both cases. Hormonal assessment was normal. Both patients underwent laparoscopic transperitoneal adrenalectomy.
Surgical time was 120 minutes and 50 minutes, respectively. One patient was discharged on postoperative day 2, and the other remained hospitalized until day 10. The latter patient required percutaneous drainage of a retroperitoneal collection. Both tumors were identified as mature cystic teratomas. No evidence was present of recurring disease in either patient.
Adrenal teratoma is rare. Laparoscopic transperitoneal adrenalectomy is a feasible, effective technique that enables excellent oncologic results. To our knowledge, this is the first report of laparoscopic adrenalectomy for pure adrenal teratoma.
Adrenal; Teratoma; Laparoscopy
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
Teratomas are a unique class of tumors composed of ecto- meso- and endodermal tissues, all foreign to the site of origin. In humans, the most common teratoma is the ovarian teratoma. Not much is known about the molecular and genetic etiologies of these tumors. Female carriers of the Tgkd transgene are highly susceptible to developing teratomas. Ovaries of Tgkd/+ hemizygous female mice exhibit defects in luteinization, with numerous corpora lutea, some of which contain central trapped, fully-grown oocytes. Genetically, Tgkd teratomas originate from mature oocytes that have completed meiosis I, suggesting that Tgkd teratomas originate from these trapped oocytes. The insertion of Tgkd 3′ of the Inpp4b gene is associated with decreased expression of Inpp4b and changes in intracellular PI3 Kinase/AKT signaling in follicular granulosa cells. Because Inpp4b is not expressed in fully-grown wild-type or Tgkd oocytes, these findings suggest that hyperactivation of the PI3K/AKT pathway caused by the decrease in INPP4B in granulosa cells promotes an ovarian environment defective in folliculogenesis and conducive to teratoma formation.
teratoma; PI3-kinase/AKT; Inpp4b; oocyte; granulosa
The association of ovarian teratoma and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a serious and potentially fatal pathology that occurs in young women and that is under-recognized. Our objectives were to analyze prevalence and outcome of this association, and increase awareness over this pathology. MEDLINE and SCOPUS for all studies published prior to November 30, 2013 including the search terms: “encephalitis” and “teratoma” were considered. All articles (119) reporting one or more cases of anti-NMDAR encephalitis and confirmed ovarian teratoma (174 cases) were included. No language restrictions were applied. Suspicious cases with no evidence of ovarian teratoma (n = 40) and another type of encephalitis also associated to ovarian teratoma (n = 20) were also considered for comparison and discussion. Data of publication and case report, surgery and outcome were collected. The distribution of published cases is heterogeneous among different countries and continents, probably in relation with level of development and health care. The mean patient age is 24 years and in the majority of cases (74%), a mature teratoma was identified, sometimes microscopically following ovarian removal or at autopsy. The clinical presentation featured psychiatric symptoms and behavioural changes, with a median delay for surgery of 28 days. Twelve women died (7%), most frequently from encephalitis-related complications. In conclusion, the association ovarian teratoma and anti-NMDAR encephalitis is relatively unknown or not reported in many countries and among gynecologists. Heightened recognition of behavioral changes, diagnosis through transvaginal ultrasound and subsequent tumor removal in addition to diagnostic confirmation through the presence of anti-NMDAR antibodies must be emphasized.
Electronic supplementary material
The online version of this article (doi:10.1186/s13023-014-0157-x) contains supplementary material, which is available to authorized users.
Anti-NMDAR encephalitis; Anti-NMDA-Receptor antibodies; Dermoid cyst; Limbic encephalitis; Ovarian teratoma
Teratomas are the commonest germ cell tumours and are most frequently found in the testes and ovary. Extragonadal teratomas are rare and mainly occur in midline structures. Uterine teratomas are extremely rare with only a few previous case reports, usually involving mature teratomas of the uterine cervix.
We report an 82-year-old lady presenting with post-menopausal bleeding. Initial investigations revealed a benign teratoma of the uterus which was removed. Her symptoms persisted and a recurrent, now malignant, teratoma of the uterine corpus was resected at hysterectomy. Six months after surgery she relapsed with para-aortic lymphadenopathy and was treated with a taxane, etoposide and cisplatin-containing chemotherapy regimen followed by retroperitoneal lymph node dissection.
In this report we discuss the aetiology, diagnosis and management of uterine teratomas, and review previous case studies.
Teratomas represent 0.5% of all intracranial tumors. These benign tumors contain tissue representative of the three germinal layers. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. The presence of a teratoma in the cavernous sinus is very rare. Congenital teratomas are also rare, especially those of a cystic nature. To our knowledge, this would be the first case report of a congenital, rapidly growing cystic teratoma within the cavernous sinus. A three-month-old boy presented with a past medical history of easy irritability and poor oral intake. A magnetic resonance image (MRI) scan of the head disclosed a large expanding cystic tumor filling the right cavernous sinus and extending into the pterygopalatine fossa through the foramen rotundum. These scans also demonstrated a small area of mixed signal intensity, the result of the different tissue types conforming to the tumor. Heterogeneous enhancement was seen after the infusion of contrast medium. However, this was a cystic tumor with a large cystic portion. Thus, a presumptive diagnosis of cystic glioma was made. With the use of a right frontotemporal approach, extradural dissection of the tumor was performed. The lesion entirely occupied the cavernous sinus, medially displacing the Gasserian ganglion and trigeminal branches (predominantly V1 and V2). The lesion was composed of different tissues, including fat, muscle and mature, brain-like tissue. The tumor was completely removed, and the pathological report confirmed the diagnosis of a mature teratoma. There was no evidence of recurrence. Despite the location of the lesion in the cavernous sinus, total removal can be achieved with the use of standard microsurgical techniques.
Benign tumors; cavernous sinus; pathology; surgery; teratoma
A 26-year-old girl was referred to us in December 2008 with progressive pelvic mass while on chemotherapy. In May 2008, she presented with large adnexal mass and high alpha-fetoprotein (AFP, 265.7 ng/mL; normal range, 0 to 10). She underwent laparoscopic right salpingo-oophorectomy with staging. Since histology was immature teratoma grade I, FIGO stage 1 she was kept on surveillance. In September 2008, she developed recurrent pelvic mass with AFP levels of 2,400 ng/mL. Three courses of chemotherapy (bleomycin-etoposide-cisplatin) were given. Post-chemotherapy AFP normalized but tumor size increased. CT-scan (abdomen-pelvis) showed a large pelvic mass with calcification specks; infiltrating the sigmoid colon and abdominal wall. With provisional diagnosis of growing teratoma syndrome she had exploratory laparotomy with excision of pelvic mass along with sigmoid colon, excision of right pelvic and subcutaneous deposits, omentectomy and sigmoid anastomosis. Left ovary, left tube and uterus appeared normal and were preserved. Histology of all masses showed mature teratoma, no immature elements. At six months follow up she is disease free and has resumed menstruation. Growing teratoma syndrome is a clinico-pathological presentation during/post-chemotherapy in malignant ovarian germ cell tumor where mature teratoma grows and requires complete surgical excision. Our case highlights the safety and adequacy concerns of laparoscopic management of malignant ovarian tumor. Literature review suggests good prospects of resumption of menses, child bearing and five year survival in case of growing teratoma syndrome.
Salpingo-oophorectomy; Growing teratoma syndrome; Immature teratoma; Malignant ovarian germ cell tumor; Chemotherapy; Laparoscopy
Intracranial mature teratoma is a rare lesion in adults. Despite several intracranial mature teratomas had been reported not to be located at the midline region, no one was found to be within cerebral falx. Herein, we reported a 37-year-old female patient with an intracranial mature teratoma confined within frontal cerebral falx. Her main complaint was intermitted headache, which could not be relieved recently by taking painkiller. Excepting for mild papilledema, we did not find positive neurological signs on physical examination. CT scanning showed it was a round homogenously hypodense lesion with hyperdense signal at its rim. MRI revealed the lesion was 3.5cm×3.6cm×4.5cm in volume, with uniformed hypointensity on T1WI, hyperintensity on T2WI and enhancement in the capsule. It was totally removed via inter-hemispheric approach, and we found the lesion was confined within the frontal cerebral falx. Postoperatively, it was proved histologically to be a mature teratoma. At three years of fellow up, neither neurological deficits nor recurrent sings on MRI was found. To our best knowledge, this is the first case of intracranial mature teratoma within cerebral falx.
Mature teratoma; Dura mater; Cerebral falx; Adults.
We discuss an extremely rare case of ganglioneuroblastoma arising within a retroperitoneal mature cystic teratoma. Radiological examinations showed a cystic tumor sandwiched between the pancreas and left kidney. Surgery was scheduled because the tumor seemed to have originated from the pancreas. En-block resection of the tumor with distal pancreatectomy, splenectomy, and left adrenalectomy was performed. In terms of macroscopic appearance, the tumor mainly consisted of a unilocular cystic mass, but the presence of a smaller, solid mass was also noted within the tumor. Histopathologic examination confirmed that the cystic mass was consistent with a mature cystic teratoma of the retroperitoneum, and in addition, a ganglioneuroblastoma was evident in the solid component. Histopathologically, the ganglioneuroblastomatous area was intimately associated with dermoid tissue of the mature cystic teratoma, thus this case was diagnosed to be a mature cystic teratoma with malignant transformation. To best of our knowledge, this is the first reported case of ganglioneuroblastoma arising in a mature cystic teratoma.
Ganglioneuroblastoma; Malignant transformation; Mature cystic teratoma; Retroperitoneum; Surgical treatment
Roughly 1% of mature cystic teratomas undergo malignant transformation. In particular, cutaneous-type adnexal neoplasms may occur in mature cystic teratomas. Sebaceous carcinomas, which arise from mature cystic teratomas, have rarely been observed, with only seven cases previously reported. Here, we present a case of a 69-year-old female who had pelvic pain for two weeks and who subsequently underwent bilateral salpingo-oophorectomy and hysterectomy. Her left ovary showed a unilocular cyst, measuring 22.0 cm in diameter, filled with sebaceous material and a few hairs. A luminally-protruding solid mass measuring 4.0 cm in diameter was also noted. Microscopic findings revealed lobular or diffusely arranged basophilic, atypical sebaceous cells connected to a typical mature cystic teratoma. Tumor cells demonstrated positive immunoreactivity for high molecular weight cytokeratin, cytokeratin 7, cytokeratin 19, epithelial membrane antigen, and carcinoembryonic antigen. Here, we present a case of sebaceous carcinoma arising from a mature cystic teratoma along with a review of previously published reports.
Sebaceous carcinoma; Sebaceous adenoma; Mature cystic teratoma; Ovary
Teratomas are characterized by containing tissue from all three germinal cell layers. Occasionally, somatic type malignancies develop within a mature cystic teratoma. We reported here a rare case of enteric type adenocarcinoma, with associated dysplastic epithelial precursor lesion, arising within a mature cystic teratoma in the retroperitoneum of a 30-year-old woman status post vaginal delivery 11 weeks earlier. The mass is 17.5 cm and cystic. A polypoid mass component measuring 4.7×4.2×2.5 cm was located inside the cystic component. Microscopically, the majority of the specimen was a mature cystic teratoma with all three germinal cell layers. The polypoid mass component was an adenocarcinoma with an adjacent dysplastic epithelial precursor lesion. The adenocarcinoma was diffusely positive for CK20 and CDX-2, and focally positive for CD7, indicating enteric differentiation. A brief review of retroperitoneal mature cystic teratomas with associated somatic type malignancy was performed.
retroperitoneal; mature cystic teratoma; adenocarcinoma.
Intraspinal mature teratomas rarely occur in adults. The present study describes an unusual case of adult intradural mature teratoma, which was completely resected. A 22-year-old female presented with an intermittent pinching pain in the lower right shank that had lasted for three months. Magnetic resonance imaging (MRI) results indicated a multicystic mass extending from the T12 to L2 vertebrae, and the tumors were certified as teratomas by a histopathological examination. The level of pain experienced by the patient was improved following the surgery. The present study also compared the literature concerning adult intradural mature teratoma, summarized the basic clinical characteristics and theory of origin of adult intradural mature teratoma and reviewed the available treatments for this disease.
intradural; intramedullary; spinal cord; teratoma; adult; case report
Although the sacrococcygeal area is the most common site for a teratoma in infants, it is a rare site for a teratoma in older patients. Most of the teratomas found in this area in adults are single mass, but in a few cases, multiple masses have been reported. The author reports on the case of an 18-year-old female patient with 3 presacral teratomas. The tumors were surgically removed via a transabdominal approach and were pathologically diagnosed as mature cystic teratomas. This case report indicates that an adult presacral teratoma can appear as multiple tumors, although it is very unusual.
Adult presacral teratoma; Multiple
Teratomas are neoplasms characterised by an abnormal growth of tissues derived from the three germinal layers. The term ‘teratoma’ is derived from the Greek root ‘teratos’, meaning monster. Germ cells develop in the embryo and subsequently become the cells that make up the reproductive system. During fetal development, these cells follow a midline path and descend into the pelvis as ovarian cells or the scrotal sac as testicular cells. The presence of germ cells in extragonadal sites is because of the failure of these cells to migrate along the urogenital ridge. Therefore, teratomas occur in order of decreasing frequency in the ovaries, testes, anterior mediastinum, retroperitoneum, sacrococcygeal region and cranium.
Liver teratomas are very rare; of the 25 hepatic teratomas described in the literature, only five have occurred in adults. The majority of the cases were in female children below the age of three, mostly arising in the right lobe of liver.
We report a case of an adult male with benign mature teratoma arising in the left lobe of liver, compressing the common bile duct and causing obstructive jaundice.
Teratomas are embryonal neoplasms that arise when totipotential germ cells escape the developmental control of primary organizers and give rise to tumors containing tissue derived from all three blastodermic layers. Teratomas have been reported to occur in various sites and organs. Teratoma of the cervical neck are relatively rare in adulthood. It usually extends from the neck to the thoracic cavity causing local mass effect. In most of the cases intrauterine diagnosis is possible by ultrasound. Because of dyspnea due to mass effect, this condition is treated promptly after birth. However cases of teratoma in adulthood with supraclavicular localization have been reported rarely in the literature. The presented case is of a 25-year-old female with a cervical mass. Histological examination revealed a benign mature teratoma. The patient has been disease free for more than nine years after surgical removal of a neck teratoma.
Teratoma; Neck; Adult; Total surgical resection; Clinically disease-free
The aim of this study was to identify predictors of viable germ cell tumor (GCT) in postchemotherapeutic residual retroperitoneal masses.
Materials and Methods:
The pertinent clinical and pathologic data of 16 male patients who underwent postchemotherapeutic retroperitoneal lymph node dissection (PC-RPLND) at King Faisal Specialist Hospital and Research Centre between 1994 and 2005 were reviewed retrospectively. It was found that all patients received cisplatin-based chemotherapy for advanced testicular GCT.
Out of the 16 male patients, 2 (13%), 8 (50%), and 6 (37%) had viable GCT, fibrosis, and teratoma, respectively. Ten (10) of the patients with prechemotherapeutic S1 tumor markers did not have viable GCT, and two of the six patients who had prechemotherapeutic S2 tumor markers have viable GCT. All tumor marker levels normalized after chemotherapy even in patients with viable GCT. Four patients had vascular invasion without viable GCT. Furthermore, four patients had more than 60% embryonal elements in the original pathology, but only 1 had viable GCT at PC-RPLND. Four of the five patients with immature teratoma had teratoma at PC-RPLND but no viable GCT; however, out of the four patients with mature teratoma, one had viable GCT and two had teratoma at PC-RPLND. Of the two patients with viable GCT, one had 100% embryonal cancer in the original pathology, prechemotherapeutic S2 tumor markers, history of orchiopexy, and no vascular invasion; the other patient had yolk sac tumor with 25% embryonal elements and 40% teratoma in the original pathology, and prechemotherapeutic S2 tumor markers.
None of the clinical or pathological parameters showed a strong correlation with the presence of viable GCT in PC-RPLND. However, patients with ≥S2 may be at higher risk to have viable GCT. Further studies are needed to clarify this.
Chemotherapy; germ cell tumor; predictor; retroperitoneal lymph node dissection
We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.
Congenital; dermis fat graft; orbital; teratoma