A giant sacrococcygeal teratoma in adult female: A case report

IntroductionSacrococcygealteratoma is very rare in adults with only few cases reported in literature. It is a slow growing benign cystic tumor with 1–2% chance for malignant transformation and may attain a huge size causing pressure effect on pelvic and intra-abdominal organs. When grows caudally, it causes discomfort, skin excoriation and cosmetic disfigurement.Presentation of caseWe present a giant, long standing Sacrococcygeal teratoma in an adult female patient which caused her significant obstructing lower gastrointestinal and urinary symptoms besides the discomfort and disfigurement of the sacral region.DiscussionSacrococcygeal teratomas are commonly present early in life but are very rare to be seen in adults with such a huge size in such a place where it interfered with patient sitting and sleeping.Diagnosis of our case was suspected by clinical examination, ultrasound and MRI while histopathology confirmed the diagnosis. It was completely excised along with the coccyx with primary wound closure and had a good postoperative recovery. Up till now, 18 months follow-up using clinical, biochemical and radiological assessment revealed no evidence of recurrence.ConclusionSacococcygeal teratoma should be considered in the differential diagnosis of large pelvic masses in adults. In the presence of an extra-corporal and deep intra pelvic extension, we recommend the posterior perineal approach for excision as it has convenient control over the mass during surgery and good cosmetic results.     

Benign sacrococcygeal teratoma with spinal canal invasion and paraplegia

Sacrococcygeal teratomas can have a wide variety of clinical presentations depending on their size, vascularity, and degree of mass effect upon adjacent structures. Intradural invasion of a sacrococcygeal teratoma is a rare variant that has generally been associated with a favorable neurologic outcome. In this report, we present the case of a neonate with paraplegia secondary to a large Altman type III sacrococcygeal teratoma with extension into the spinal canal. The neoplasm was completely removed using a combined anterior and posterior approach after preoperative embolization of the lateral sacral arteries. Pathology showed a mature teratoma. The patient remains paraplegic without evidence of tumor recurrence at 1 year of age.     

Congenital-infantile fibrosarcoma masquerading as sacrococcygeal teratoma

Infantile fibrosarcoma is rare and represents less than 1% of all childhood cancers. Commonly, it arises in the limbs followed by the trunk and head and neck. We report a rare case of infantile fibrosarcoma masquerading as sacrococcygeal teratoma in a newborn. The literature on the subject is also reviewed.     

Damage control operation for massive sacrococcygeal teratoma

Surgical resections of massive sacrococcygeal teratomas (SCTs) carry significant risk due to baseline hemodynamic instability and the potential for significant hemorrhage. In this case report, a fetus with sacrococcygeal teratoma developed high-output cardiac instability at 32 weeks' gestation. After urgent cesarian delivery, a damage-control operation using Teflon-pledgeted mattress sutures allowed for hemodynamic control of bleeding into the tumor. One week later, after subsequent fluid resuscitation and stabilization, an elective staged resection of the complete mass including the pelvic portion was performed. The patient was discharged home on postoperative day 30 and was doing well at her most recent clinic visit at 30 months of age.     

Contouring buttock reconstruction after sacrococcygeal teratoma resection

Purpose

Massive sacrococcygeal teratomas are typically resected and closed in a “chevron” fashion. The resultant scar may leave protuberant “dog ears” and extend across and below the infragluteal creases down onto the posterior thighs, causing undesirable buttock deformity. Given the redundant skin often available, the authors sought to develop a closure technique to minimize deformity and unpleasant scars.

Methods

At the time of resection of 2 sacrococcygeal teratomas, attention was directed to minimizing redundant skin, restoring normal buttock contour, and avoiding scars crossing the infragluteal crease. After properly securing the anal location, serial polygonal skin excisions were performed, working the excess tissue centrally rather than peripherally, leaving 2 right-angled scars on each buttock.

Results

Each infant underwent successful reconstruction with a normal buttock contour without redundancy. All scars on the buttocks can be easily covered by bathing attire.

Conclusions

The excess skin expanded by large sacrococcygeal teratomas affords an opportunity to apply straightforward tissue rearrangement principles to reconstruct the buttocks with a normal contour and hidden scars.     

Half-life of alpha-fetoprotein in neonatal sacrococcygeal teratoma

Background

Alpha-fetoprotein (AFP) is useful as a tumor marker for sacrococcygeal teratoma (SCT). We investigated the half-life of AFP in SCT.

Gait analysis in patients operated on for sacrococcygeal teratoma

Background

Long-term follow-up of sacrococcygeal teratoma (SCT) is well established; however, little is known about the effects of extensive surgery in the pelvic and perineal region, which involves disruption of muscles providing maximal support in normal walking.

Methods

Thirteen patients operated on at birth for SCT with extensive muscle dissection underwent gait studies with a Vicon 3-D motion analysis system with 6 cameras. Results were compared with 15 age-matched controls. Statistical analysis was performed with Mann-Whitney test; correlations were sought with Spearman’s correlation coefficient.

Results

All subjects were independent ambulators, and no statistically significant differences were seen in walking velocity and stride length. However, in all patients, toe-off occurred earlier (at 58% ± 1.82% of stride length) than controls (at 65.5% ± 0.52%; P < .05). On kinetics, all patients exhibited, on both limbs, a significant reduction of hip extensory moment (−0.11 ± 0.11 left; −0.16 ± 0.15 right v 1.19 ± 0.08 Newtonmeter/kg; P < .05) and of ankle dorsi/plantar moment (−0.07 ± 0.09 right; −0.08 ± 0.16 v −0.15 ± 0.05 Nm/kg, p < 0.05). Knee power was also significantly reduced (0.44 ± 0.55 right, 0.63 ± 0.45 left v 0.04 ± 0.05 W/kg), whereas ankle power was increased (3 ± 1.5 right; 2.8 ± 0.9 left v 1.97 ± 0.2 W/kg; P < .05). No statistically significant correlation was found between tumor size and either muscle power generation or flexory/extensory moments.

Conclusions

Patients operated on for SCT exhibit nearly normal gait patterns. However, this normal pattern is accompanied by abnormal kinetics of some ambulatory muscles, and the extent of these abnormalities appears to be independent of tumor size. A careful follow-up is warranted to verify if such modifications are stable or progress over the years, thereby impairing ambulatory potential or leading to early arthrosis.     

Prenatally diagnosed sacrococcygeal teratoma: a prognostic classification

Purpose

The objective of this study is to describe a prognostic classification for prenatally diagnosed sacrococcygeal teratoma (SCT).

Methods

Charts from 44 fetuses were reviewed. Three groups were defined as follows: group A—tumor diameter less than 10 cm, absent or mild vascularity and slow growth; group B—diameter 10 cm or greater, pronounced vascularity or high-output cardiac failure and fast growth; group C—diameter 10 cm or greater, predominantly cystic lesion with absent or mild vascularity and slow growth.

Results

Size at diagnosis, growth rate, and vascularity were higher in group B. Gestational age at delivery was lower in group B. Eleven of 21 died in the perinatal period in group B and none in groups A and C. In group C, drainage or shunting of the SCT has been performed in 6 of 10 cases.

Conclusions

Group A is associated to good maternal and perinatal outcome, as well as group C, although shunting or drainage of the SCT could be necessary. Large fast-growing SCT with rich vascularity is associated with a higher perinatal mortality and morbidity than smaller lesions with mild vascularity.     

Type IV sacrococcygeal teratoma causing urinary retention: a rare presentation

Sacrococcygeal teratoma is a rare fetal neoplasm usually diagnosed antenatally and considered benign with no functional impairment. Nevertheless, fetuses with this malformation are at risk of significant perinatal morbidity and mortality, usually implying a malignant component. Management is primarily surgical, with a generally good prognosis. Herein, the authors present a rare and unusual presentation of a type IV sacrococcygeal teratoma as urinary retention in a 7-day-old infant. The diagnostic workup and subsequent surgical treatment are described. The nature of the tumor and the treatment is discussed, emphasizing the need for urgent surgical treatment because of bilateral hydronephrosis that failed to resolve after inserting a urinary catheter.     

The incidence of associated abnormalities in patients with sacrococcygeal teratoma

Background

Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors.

Two cases of unusual urethral complications after resection of sacrococcygeal teratoma

This report describes 2 rare complications of a sacrococcygeal teratoma leading to urethral obstruction in 1 case and midurethral necrosis in the other. The anomalies appear to be based on pressure necrosis of the urethra by mechanical compression between the symphysis pubis and the teratoma.     

Study of the factors associated with recurrence in children with sacrococcygeal teratoma

Purpose

The aim of this study was to explore effects of (1) histological involvement of resection margins with microscopic residue, (2) incomplete removal of coccyx, and (3) tumor spillage on recurrence and on survival in children operated upon for sacrococcygeal teratoma (SCT).

Methods

Retrospective review of 70 patients treated between 1960 and 2003.

Results

Fifty-four girls and 16 boys presented with SCT diagnosed prenatally (12), at birth (37), or later (21). Thirty-six percent of tumors were Altman type I, 27% type II, 18% type III, and 18% type IV. Histologically, mature teratoma was observed in 48 patients, immature teratoma in 11, yolk sac tumor (YST) in 9, embryonal carcinoma in one, and mixed tumor in one. Eighty-four percent of patients solely underwent surgical extirpation. Six (8.5%) patients died. However, mortality for the group of 42 patients treated during the past 15 years was as low as 2.5%. Tumor recurrence was observed in 5 patients, 2 of whom died. Of 3 patients with initially mature teratoma, 1 showed local immature recurrence and 2 malignant recurrences. One of the latter died. Of 2 patients with initially immature teratoma grade I, one relapsed with a benign lesion and one with YST leading to death. Possible eliciting factors had been demonstrated in 3 patients. Histological analysis of resection margins showed tumoral involvement in 11 patients (and also in one patient after resection of a recurrent tumor). Only one of those with YST focus in the resection margin showed recurrence. Intraoperative tumor spillage presented in 2 patients, who both died of metastatic disease. Spillage of tumoral cyst fluid occurred in 6, none developed recurrence. One of 5 patients whose coccyx had not been removed died of metastatic disease. One with immature teratoma developed a benign recurrent tumor. The other 3 showed no recurrence.

Conclusions

Microscopic involvement of the resection margins of mature or immature SCT is rarely associated with recurrence, provided there are no YST foci in the resection margins. A conservative attitude then appears to be justified. Spillage of cyst fluid was never associated with recurrence, unlike spillage of tumor and absence of removal of coccyx.     

Laparoscopic ligation of the median sacral artery before resection of a sacrococcygeal teratoma

To minimize the risk of exsanguinating hemorrhage, the authors describe a technique of laparoscopic ligation of the median sacral artery before posterior sagittal resection of type I sacrococcygeal teratoma (SCT). Two female infants with antenatally diagnosed SCT underwent postnatal evaluation and preoperative imaging and were taken to the operating room. In both patients, pneumoperitoneum was established via an epigastic 5-mm trocar. Two additional trocars were inserted in the right and left lower quadrants. The peritoneal reflection was opened to the right of the sigmoid colon, and the presacral space was explored. A large median sacral artery was identified easily, isolated, and divided. The children then were placed in a prone position, and the tumors underwent en bloc resection via a Chevron incision with minimal blood loss. The laparoscopic portion of the procedure was performed in an average of 15 minutes. This is the first report of laparoscopic ligation of the median sacral artery before posterior resection of a sacrococcygeal tumor in an infant. This technique can be performed easily with minimal morbidity. Division of this artery is a logical preventative measure and may reduce the risk of hemorrhage during operative resection.     

A case of neonatal neuroblastoma mimicking Altman type III sacrococcygeal teratoma

The authors report a case of neonatal neuroblastoma mimicking Altman type III sacrococcygeal teratoma. A newborn male infant delivered after a normal pregnancy was found to have an extremely large sacrococcygeal mass. Imaging studies strongly suggested Altman type III sacrococcygeal teratoma. On the 10th day after the birth, the tumor was incompletely removed at surgery. Histopathologic examination of the tumor showed neuroblastoma. The tumor disappeared completely after chemotherapy. One year after diagnosis, no local recurrence or metastasis had been detected. To the authors' knowledge, this is the first case of neonatal neuroblastoma mimicking Altman type III sacrococcygeal teratoma.     

Enhancing safety of laparoscopic vascular control for neonatal sacrococcygeal teratoma

Life-threatening bleeding is a hazard of major tumor excision in children. However, fatalities from inadvertent arterial ligation should not be overlooked. Sacrococcygeal teratoma is the commonest neonatal tumor. Laparotomy to ligate the median sacral artery has been used to preempt potentially fatal resectional bleeding. Use of laparoscopy to achieve the same is an evolving technique, with only 7 neonatal cases described. As such, the Idea, Development, Exploration, Assessment, Long-term study (IDEAL) guidelines on surgical innovation recommend case reports addressing proof of concept, technical factors and safety tips. Fortunately, mistaken arterial division is so far unreported during laparoscopic median sacral artery ligation. However, as uptake widens, anatomical distortion by tumor and surgeon disorientation at endosurgery are risk factors for even such inconceivable complications. We report a successful case of laparoscopic vascular control for neonatal sacrococcygeal teratoma and demonstrate an observation that serves as a useful safety check for this procedure (as well as the open alternative).     

Long-term functional sequelae of sacrococcygeal teratoma: a national study in The Netherlands

Background

Long-term functional sequelae after resection of sacrococcygeal teratoma (SCT) are relatively common. This study determines the incidence of these sequelae associated clinical variables and its impact on quality of life (QoL).

Patients and methods

Patients with SCT treated from 1980 to 2003 at the pediatric surgical centers in the Netherlands aged more than 3 years received age-specific questionnaires, which assessed parameters reflecting bowel function (involuntary bowel movements, soiling, constipation), urinary incontinence, subjective aspect of the scar, and QoL. These parameters were correlated with clinical variables, which were extracted from the medical records. Risk factors were identified using univariate analysis.

Results

Of the 99 posted questionnaires, 79 (80%) were completed. The median age of the patients was 9.7 years (range, 3.2-22.6 years). There were 46% who reported impaired bowel function and/or urinary incontinence (9% involuntary bowel movements, 13% soiling, 17% constipation), and 31% urinary incontinence. In 40%, the scar was cosmetically unacceptable. Age at completion of the questionnaire, Altman classification, sex, and histopathology were not risk factors for any long-term sequelae. Size of the tumor (>500 cm³) was a significant risk factor for cosmetically unacceptable scar (odds ration [OR], 4.73; confidence limit [CL], 1.21-18.47; P = .026). Long-term sequelae were correlated with diminished QoL.

Conclusion

A large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables.     

Preoperative embolization of giant sacrococcygeal teratoma in a premature newborn

Resection of a large vascular sacrococcygeal teratoma (SCT) in a newborn has the potential to be a fatal procedure caused by hemolysis, rupture, or bleeding of the tumor. Usually, most blood supply of an SCT is derived from the middle sacral artery. As soon as these arteries have been ligated, further blood loss is minimal. There is only one previous presentation about preoperative embolization of these arteries. We present a case in which the feeding arteries of a giant SCT were embolized in an infant born at 30 weeks and 3 days of gestation. Although bleeding during the surgery was minimal, continuous need of transfusions and life-threatening hyperkalemia created severe problems during surgery, until tumor resection was completed. This is the smallest reported patient in whom SCT was preoperatively treated by embolization.     

Monochorionic twin gestation complicated by a sacrococcygeal teratoma with hydrops--optimal timing of delivery allows for survival of the unaffected twin

Sacrococcygeal teratomas are the most common tumors found in newborns and have a favorable outcome when diagnosed late in gestation. Solid, highly vascularized tumors diagnosed early in gestation have a higher incidence of hydrops fetalis and fetal demise. The following is a case report of a monochorionic twin gestation complicated by a solid highly vascular sacrococcygeal teratoma and the development of hydrops in the affected twin. In this case, serial ultrasound evaluations and timing of delivery optimized the outcome of the unaffected twin.     

Benign cervical teratoma in an adult: Report of a case

Cervical teratomas are rarely encountered in adults. We report herein the case of a 21-year-old woman who was admitted to our hospital for surgical treatment of a neck tumor, 7.5×4.5×2.7 cm in size, located in the left lower pole of the thyroid. Ultrasonography (US) and computed tomography (CT) revealed a multicystic tumor. The levels of carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA 19-9), and squamous cell carcinoma-related antigen (SCC) in the cystic fluid were extremely elevated in contrast to the normal levels found in the serum. The tumor was completely excised and histological examination revealed it to be composed of elements derived from the three germ layers, confirming a diagnosis of benign cystic teratoma.