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
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.
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.
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.
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
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
Teratoma is a rare developmental neoplasm that arises from totipotential tumor stem cells. Head and neck teratomas constitute about 10% of all cases. Only two cases of mature teratoma of the nasal septum have previously been documented in the world literature.
We present a case of histologically confirmed mature teratoma arising from the nasal septum in an eighteen month old Nigerian female who presented with a history of noisy breathing associated with recurrent rhinorrhea since birth. Physical examination revealed obstruction of the right nasal cavity by a pale fleshy mass. She underwent a total surgical excision and to date, after thirty one months follow-up, she is free from recurrence.
The prognosis for benign teratoma of the nasal septum is good following total surgical excision.
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
Mature teratoma is the most common primary germ cell tumor in the mediastinum. On rare occasions, cystic teratomas rupture in adjacent structures, such as pleural space, pericardium, lung or tracheobronchial tree. We present a case of a mediastinal mature cystic teratoma in 16-year-old female with complex rupture into the lung, bronchus and skin. Mature mediastinal teratoma fistulized to the skin has not been previously described.
Mediastinal teratoma with airway communication; Skin fistula; Hemoptysis
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.
This case report illustrates a rare case of teratoma of the mediastinum which was continuous to the pericardium and caused extrinsic compression to the right atrium.
A 22-year-old Caucasian man with no complaints or comorbid conditions presented to our hospital with obliteration of the right cardiophrenic sinus by a mass. A non-invasive investigation demonstrated a tumoral mass which was continuous to the pericardium and caused extrinsic compression to the right atrium. The clinical suspicion was a pericardial or bronchogenic cyst. Surgical and anatomopathologic findings led to the diagnosis of a mature cystic teratoma with atrophic thymic tissue at the external teratoma surface.
We present an original report of a mature teratoma causing obliteration of the right cardiophrenic sinus with extrinsic heart compression. The diagnosis of this tumor is very difficult through non-invasive investigation.
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
Benign cystic teratoma is recognized as one of the most common tumors in women during the reproductive age and frequently is treated by pelviscopic operation. Malignant transformation of a benign cystic teratoma is a rare event, and adenocarcinoma is extremely rare, and distinguishing this malignant change from benign disease preoperatively is nearly impossible even by the use of radiological imaging or various tumor markers. Therefore, patients should be informed that if a laparoscopic cystectomy is undertaken, a prompt second staging operation should be performed if the definitive pathology reveals an unexpected malignancy. We present a case with thyroid papillary carcinoma of follicular variant arising from mature cystic teratoma removed by laparoscopic salpingo-oophorectomy followed by staging laparotomy. We briefly reviewed literatures with regard to malignant transformation of a benign cystic teratoma.
Mature cystic teratoma; Malignant transformation; Adenocarcinoma; Tumor marker
Teratomas of the sacrococcygeal area are usually diagnosed in infancy and are rarely seen in adults.
We report the case of a 36-year-old Greek woman experiencing chronic constipation due to a benign presacral teratoma. Imaging examinations showed a pelvic mass without evidence of malignancy. An ovoid tumour with a maximum dimension of 6 cm was surgically removed. A histologic examination revealed a mature cystic teratoma. Two years after surgery, the patient is well, with no evidence of recurrence and no constipation.
Sacrococcygeal teratomas are rare in adults. A high index of suspicion is important in making an early diagnosis. Rectal examination and radiologic evaluation are also valuable.
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
Teratomas are the most common congenital tumours, but teratomas of the nasopharynx are rare in neonates. The present report concerns a premature 30-week-old infant girl with an extensive nasopharyngeal teratoma protruding from the oral cavity. The tumour completely obstructed the airway and necessitated immediate intubation. The tumour was successfully resected directly via the transoral route without needing external incisions. Histological examination of the specimen revealed it to be a mature teratoma. The management and differential diagnosis is discussed, accompanied by a review of the literature.
Of a total of 307 patients treated with chemotherapy for advanced non-seminomatous germ-cell testicular tumours between 1976 and 1983, 73 (23.8%) had masses excised after treatment. Resected tissue showed residual malignancy in 16 (22%), fibrosis and necrosis in 25 (34%) and differentiated (mature teratoma) in 32 (44%). Of the 16 patients with tumour only 7 (44%) are alive and disease-free compared with 21/25 (84%) and 27/32 (84%) for fibrosis/necrosis and differentiated teratoma respectively. In addition to histological evidence of residual tumour, elevated serum markers at the time of surgery and/or incomplete excision of residual masses were adverse prognostic features. Of 12 patients with differentiated teratoma or fibrosis who had incomplete resections or densely adherent masses excised with difficulty, 7 subsequently relapsed. The majority of differentiated teratoma patients (75%) had evidence of differentiation in their primary tumours; 88% showed cystic change in metastases and almost one-third showed an increase in the size of metastases during chemotherapy. The data suggest that post-chemotherapy surgery may have a therapeutic as well as a diagnostic role and that complete excision of residual disease should be attempted even if resection at one site has shown either fibrosis or differentiated teratoma. The significance of these findings in relation to treatment induced differentiation is discussed.
A 48-year-old male who presented with an enlarged right scrotum was diagnosed with malignant transformation of testicular teratoma. Physical examination revealed a right scrotal mass of hard consistency with no inguinal lymphadenopathy. Since prepuberty, his right testis had been larger than the left one, with no pain or tenderness. Computed tomography and bone scan revealed retroperitoneal lymphadenopathy and multiple bone metastases. Right orchiectomy was performed immediately, and a pathological examination revealed a mature teratoma associated with adenocarcinoma, showing signet ring cell differentiation. Cisplatin-based combination chemotherapy was administered; however, the metastatic lesions progressed, and the patient succumbed to the disease after 15 months. Only a few cases of primary malignant transformation of teratoma in the testis have been reported, and this is the first case report of primary malignant transformation of teratoma in the testis with signet ring cell-type differentiation.
adenocarcinoma; testis; teratoma
Primary retroperitoneal teratomas involving adrenal glands are exceedingly uncommon accounting for only 4% of all primary teratomas. They are more common in childhood and rarely occur in adults. Only a very few case reports have been documented in literature so far. Herein, we report a mature (benign) cystic retroperitoneal teratoma in the region of left adrenal gland in a 22-year-old otherwise healthy male patient who presented with a 1-month history of left flank pain. In addition, a literature review on teratomas is included.
Background. Although some mature cases of teratomas have recently been described in the cervix, they are not commonly found in the uterus, especially in immature forms. An immature uterine teratoma relapsing after surgery as malignant neuroepithelioma has never been reported in the literature. Case Presentation. We describe a case of immature teratoma which occurred primarily in the uterus in a 56-year-old female. Treatment consisted of total simple hysterectomy. Three months after surgery, the patient relapsed with voluminous pelvic mass and was treated with bleomycin, etoposide, and cisplatin-containing chemotherapy regimen. Conclusion. In this report and according to the pertinent literature, clinical and pathological features and management of uterine immature teratomas are discussed. The mainstay of treatment is surgery. The prognosis of this unusual disease remains uncertain.
We report a patient who has maintained a regular menstrual cycle despite undergoing cystectomy and chemotherapy for contralateral recurrence of ovarian immature teratoma with gliomatosis peritonei. We initially performed a fertility-sparing right salpingo-oophorectomy, omentectomy and peritoneal biopsy for immature teratoma with gliomatosis peritonei, with adjuvant chemotherapy; we performed a left ovarian cystectomy and peritoneal biopsy for mature cystic teratoma with gliomatosis peritonei 16 months after the first surgery, a fertility-sparing left ovarian cystectomy and peritoneal biopsy for contralateral recurrence of ovarian immature teratoma with gliomatosis peritonei 60 months after the first surgery, and a left ovarian cystectomy and peritoneal and external iliac lymph node biopsy for endometrial cyst with gliomatosis peritonei 71 months after first surgery. The peritoneal gliomatosis lesions gradually decreased through the 4 surgeries over 8 years. The patient has maintained a regular menstrual cycle and currently shows no evidence of disease.
immature teratoma; conservative surgery; gliomatosis; recurrence; ovary
The retroperitoneum is an uncommon location for teratoma in adults. The current study presents the case of a rare giant primary retroperitoneal teratoma in a 55-year-old female. The clinical manifestations, diagnosis and surgical treatment procedure of this case are retrospectively reviewed. The patient presented with a complaint of an abdominal palpable mass and fullness for 1 month. The patient suffered a massive hemorrhage during the first exploratory laparotomyand only a small section of the tumor was resected. Pathology revealed a mature retroperitoneal teratoma. Eleven months after the first surgery, the tumor was resected successfully at the second laparotomy. The surgical specimen was a large tumor mass measuring 22×18×10 cm in size and weighing 6 kg. At follow-up, the patient was in a stable condition. This case study highlights the importance of imaging for the development of a pre-operative strategy and performing a safe surgical excision in giant retroperitoneal teratoma cases.
retroperitoneal teratoma; primary; adult