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Background
Sacrococcygeal teratomas are a rare subset of germ cell neoplasms occuring predominately in infants and children. Most of the cases are primary. They arise within the retroperitoneum, sacrococcygeal region, or involve both compartments.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION 75-90% occur in females
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES PRIMITIVE MYXOID MESENCHYMAL TUMOR OF INFANCY
- Primitive Myxoid Mesenchymal Tumor of Infancy: A Clinicopathologic Report of 6 Cases.
Alaggio R, Ninfo V, Rosolen A, Coffin CM.
*Department of Oncology and Surgery, Section of Pathology daggerDepartment of Pediatrics, University of Padova, Padova, Italy double daggerDepartment of Pathology, Primary Children's Medical Center and University of Utah School of Medicine, Salt Lake City, UT.
Am J Surg Pathol. 2006 Mar;30(3):388-394. Abstract quote
Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma.
In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis. PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass. All patients were otherwise healthy. The tumors occurred on the trunk, extremities, and head and neck. Grossly, the tumors were nonencapsulated and had a multinodular appearance with focal infiltrative growth, a white fleshy cut surface, and a tumor diameter ranging from 2 to 15 cm.
Histologically, a diffuse growth of primitive spindle, polygonal, and round cells occurred in a myxoid background. The tumor cells were arranged in a vaguely nodular pattern with peripheral collagenized stroma, higher cellularity at the periphery, and a delicate vascular network in the background.
Immunohistochemically, the tumors displayed diffuse reactivity for vimentin and no reactivity for smooth muscle actin, muscle specific actin, desmin, S-100 protein, or myogenin. Electron microscopy documented a poorly differentiated fibroblastic proliferation. Four cases tested negative for the ETV6-NTRK3 gene fusion by RT-PCR. One tumor had a complex karyotypic abnormality with rearrangements involving chromosomes Y, 9, and 3. Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy. One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up.
The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS Surgery 1968;64:834-846
Critical age cutoff is 2 months with tumors below this age group almost always benign and tumors above this age group usually malignant
RECURRENCE Mature teratomas excised in early life may recur with similar histology, immature elements, or malignant germ cell tumor
Rarely, an adenocarcinoma may occur
Malignant benign neonatal sacrococcygeal teratoma.Bilik R, Shandling B, Pope M, Thorner P, Weitzman S, Ein SH.
Department of Surgery, University of Toronto, Hospital for Sick Children, Ontario, Canada.
J Pediatr Surg 1993 Sep;28(9):1158-60 Abstract quote Thirty-six patients with benign neonatal sacrococcygeal teratoma (SCT) were treated in our medical center from 1972 to 1990. Mean gestational age was 38.6 +/- 3.3 weeks with a mean birth weight of 3,484.0 +/- 938.5 g. Twenty-nine patients (89%) were females.
The majority of the tumors (75%) contained cystic components and 96% were Altman classification I and II. The initial surgical removal of the SCT (including the coccyx) was carried out during the first 7 days of life. Six patients (22%) developed recurrence of the tumor. Three were benign and reappeared locally after 12 +/- 3 months and were reexcised. The mean serum alpha-fetoprotein level in this group was 13 +/- 1 g/L. The malignant recurrence (all originally reported as being mature benign SCT) appeared at 20.3 +/- 1.5 months and had markedly elevated serum alpha-fetoprotein levels (7,320 +/- 4,630 micrograms/L). All the patients in this group had multimodal therapy including complete excision of the recurrent tumor.
We conclude that SCT, although histologically benign, has an alarming potential to recur either as a benign or malignant tumor during the first 2 years of life. Close follow-up for at least 3 years (frequent examination, serum alpha-fetoprotein, and diagnostic imaging) is recommended for all patients who had undergone excision of SCT in the newborn period.
SURVIVAL
Sacrococcygeal teratoma: clinical course and prognosis with a special view to long-term functional results.Schmidt B, Haberlik A, Uray E, Ratschek M, Lackner H, Hollwarth ME.
Department of Pediatric Surgery, University Hospitals of Graz, Austria.
Pediatr Surg Int 1999;15(8):573-6 Abstract quote From 1976 to 1995, 23 children, 4 boys and 19 girls, were treated at our department for sacrococcygeal teratomas (SCT).
Their records were analyzed retrospectively, considering age at operation, histopathology, recurrences, and long-term evolution. One died on the 1st day of life following tumor rupture with hemorrhagic shock without surgical intervention. All others were operated upon at a mean age of 4.2 days for those 19 (= 82%) who were diagnosed in the neonatal period and whose histology proved benign. In the remaining 3 children, in whom tumor manifestation did not occur before 11 months, 13 months, and 10 years of age, respectively, histopathologic evaluation revealed 2 carcinomas and 1 yolk-sac tumor, and all 3 recurred.
Overall, 5 patients died, the 1 mentioned above, 1 due to volvulus after laparotomy, and 1 from multiple associated congenital malformations. Two deaths were related to malignancy, whereby only 1 was a malignant teratoma diagnosed at the original operation. Eight children had recurrences, 2 were benign and 6 malignant, with 3 of the latter having been graded benign on histology of the primary tumor. Of the 18 surviving patients, 17 (93.5%) returned for clinical review following a standardized protocol. The average interval from the primary surgery was 12.3 years (range 3.5-22 years). Four had malignant tumors with a recurrence-free period of from 9 to 14 years; 5 (29.4%) had urinary or anorectal functional impairment. One child with a patulous anus presented with fecal soiling. Two reported nocturnal enuresis, 1 associated with perineal anesthesia. One had a neurogenic bladder with overflow voiding and bilateral third-degree vesicoureteral reflux. Second-degree reflux was found in the last patient.
We conclude that follow-up after surgery for SCT should not only search for tumor recurrence but include the diagnosis and treatment of possible secondary urinary and/or fecal incontinence.
Sacrococcygeal teratoma: a series of 19 cases with long-term follow-up.Uchiyama M, Iwafuchi M, Naitoh M, Yagi M, Iinuma Y, Kanada S, Takeda M.
Department of Pediatric Surgery, Niigata University School of Medicine, Japan.
Eur J Pediatr Surg 1999 Jun;9(3):158-62 Abstract quote A series of 19 cases of sacrococcygeal teratoma (SCT) with follow-up of 5 to 25 years is presented.
Twelve patients were neonates, age 0 to 26 days (5 immature teratomas and 7 mature teratomas, representing 3, 6, 2, and 1 Altman's type I, II, III, and IV tumors, respectively), four were infants, age 1 to 6 months (all mature teratomas, representing 1, 1, and 2 Altman's type I, II, and IV tumors), and 3 were children, age 1 to 4 years (all malignant teratomas, all Altman's IV tumors). Eight babies were delivered by elective caesarean section (CS). Though the mean gestational age at CS was 34.3 weeks in our series, we now believe that CS often must be performed earlier, depending on a tumor size or fetal condition.
Eleven neonates and 4 infants were operated upon using a sacral approach in 10 and an abdominosacral approach in 5, and all survived. However, 4 patients had neurogenic bladder and were treated by urinary catheterization or vesicostomy for 2 to 5 years after surgery. Postoperative urogenital sequelae are seen in patients with a large tumor, urethral compression, urinary retention, or edema of the lower body.
Malignant tumors usually had metastasized by the time of diagnosis, but the prognosis for outcome has been improved following surgery and combination chemotherapy.
Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group.Rescorla FJ, Sawin RS, Coran AG, Dillon PW, Azizkhan RG.
J.W. Riley Hospital, Indianapolis, IN 46202, USA.
J Pediatr Surg 1998 Feb;33(2):171-6 Abstract quote BACKGROUND/PURPOSE: Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the effect of therapy on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT).
METHODS: The authors conducted a retrospective review of children with SCT treated at 15 Childrens Cancer Group institutions from 1972 to 1994.
RESULTS: One hundred twenty-six children presented with SCT diagnosed prenatally (n = 32), at birth (n = 79), or later in infancy (n = 15). For neonates, complete resection was performed except in two babies with lethal associated defects. All others (n = 15) underwent resection at the age of diagnosis. Six had a sacral mass identified at birth but had delayed surgery (1.5 to 11 months) and of these, two were malignant. Resection was via sacral (n = 96) or abdominosacral (n = 28) approach. Histology showed mature teratoma (MT, 69%), immature teratoma (IT, 20%), or endodermal sinus tumor (EST, 11%) at presentation. Seven neonates (5.6%) died of perioperative complications, whereas the remaining 117 were available for long-term follow-up. Between 6 and 34 months postresection, recurrent disease developed in 9 of 80 MT patients (11%) followed-up for a mean of 5 years. Recurrent disease was MT (n = 2) and EST (n = 7). The recurrent EST patients were treated with adjuvant chemotherapy. Six are alive with mean follow-up of 114 months, whereas one with metastatic disease was lost to follow-up. Recurrence (MT) developed in only 1 of 24 IT patients, and all are alive and well at mean follow-up of 39 months. Patients presenting with EST (n = 13) underwent excision, with two dying from non-EST causes. Six EST patients received no chemotherapy, with two of the six (33%) experiencing recurrence within 11 months and both disease free after salvage chemotherapy. The remaining five EST patients received adjuvant chemotherapy; four are alive and one died of metastatic disease. Of the 18 EST patients followed-up after resection (presentation, 11, recurrent teratoma, 7), 16 (89%) are free of disease with a mean follow-up of 91 months.
CONCLUSIONS: (1) Benign teratomas have a significant recurrence rate mandating close follow-up for more than 3 years. (2) Surgical resection alone is adequate therapy for nonmetastatic malignant tumors. (3) Survival for malignant lesions with metastases is excellent with modern chemotherapy.
TREATMENT Am J Surg 1985;150:373-375
Total excision must include the coccyx to prevent recurrence
Sacrococcygeal teratoma.Wakhlu A, Misra S, Tandon RK, Wakhlu AK.
1/147 Vivek Khand, Gomti Nagar, Lucknow - 226 010, UP, India
Pediatr Surg Int 2002 Jul;18(5-6):384-7 Abstract quote This retrospective study details our experience regarding 72 patients with sacrococcygeal teratoma treated over a period of 17 years.
The sex incidence was nearly equal, but there was a high proportion of Altmann type IV tumors. A preliminary colostomy before combined abdominosacral excision of large type III and IV lesions reduced morbidity. Sixty-six percent of the patients presented beyond the neonatal period; 14 had been treated elsewhere for bowel/urinary obstruction. Imaging studies included radiography, abdominal ultrasound, computed tomography, and magnetic resonance imaging (after 1995). In 60 patients the tumor was excised via the sacral route, 11 had a preliminary colostomy, and 1 had a vesicostomy. Eight children (5 with malignant lesions) required abdominosacral excision. After 1990, serial estimation of serum alpha-fetoprotein (AFP) was used to monitor tumor recurrence. There were 34 male and 38 female patients (age range 3 days-12 years); 47 had benign tumors, of which 42 were excised through the sacral route.
Three patients underwent a preliminary colostomy and abdominosacral excision of the tumor with subsequent colostomy closure. There were 4 deaths in this group; no recurrence was seen in the surviving children with benign tumors. Twenty-five patients had malignant teratomas. In 18 of these the tumor was excised via the sacral route and 5 underwent abdominal-sacral excision. Eight had a preliminary colostomy and chemotherapy followed by excision of the residual tumor and colostomy closure. None of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery. One patient is currently on preoperative chemotherapy and another developed recurrence of the tumor.
The overall follow-up ranged from 3 months to 8 years; there has been no complaint of functional neurological deficit in any of the patients. As intrapelvic tumors tend to have a delayed diagnosis, this can be avoided by performing a rectal examination. There should be no recurrence after excision of a benign teratoma.
Cisplatinum-based chemotherapy has improved the survival of patients with malignant tumors.
Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89.Gobel U, Schneider DT, Calaminus G, Jurgens H, Spaar HJ, Sternschulte W, Waag K, Harms D.
Department of Pediatric Hematology and Oncology, Children's Hospital, Heinrich-Heine-University, Medical Center, Dusseldorf, Germany.
J Clin Oncol 2001 Apr 1;19(7):1943-50 Abstract quote PURPOSE: To evaluate a multimodal approach including surgery and cisplatinum chemotherapy for treatment of children with malignant sacrococcygeal germ cell tumors (GCT) and to compare adjuvant and neoadjuvant strategies in advanced tumors.
PATIENTS AND METHODS: Between 1983 and 1995, 71 patients with malignant sacrococcygeal GCT were prospectively enrolled onto the German protocols for nontesticular GCT Maligne Keimzelltumoren 83/86 and 89. Five patients who received no chemotherapy (n = 2) or nonplatinum chemotherapy (n = 2) or who did not undergo tumor resection (n = 1) were excluded from this analysis. Among the 66 patients analyzed were 14 boys and 52 girls. The median age was 17.4 months (range, 7 months to 119 months). Median follow-up was 79 months (range, 4 months to 145 months).
RESULTS: Fifty-two patients presented with locally advanced stage T2 tumors, and 30 patients had distant metastases at diagnosis. Patients received a median of eight cycles (range, four to nine cycles) of cisplatinum-based chemotherapy. Thirty-five patients underwent tumor resection at diagnosis and received adjuvant cisplatinum-based chemotherapy (group A). Thirty-one patients received up-front chemotherapy followed by delayed tumor resection (group B). Group B included more metastatic tumors than group A (group B, 19 of 31 patients; group A, 11 of 35 patients, P =.01). Preoperative chemotherapy facilitated complete tumor resections (group B, 20 of 31 patients; group A, five of 35 patients, P <.001) and avoided second-look surgery. Metastases at diagnosis and completeness of the first attempt of tumor resection were significant prognostic predictors; however, metastases were not predictive for patients treated with up-front chemotherapy. At 5 years follow-up, event-free survival was 0.76 +/- 0.05 (50 of 66 patients), and overall survival was 0.81 +/- 0.05 (54 of 66 patients). Four patients died as a result of therapy-related complications, and eight patients died of their tumors. Patients with locally advanced and metastatic tumors (T2b M1) fared better with neoadjuvant treatment [overall survival: 0.83 +/- 0.09 (16 of 19 patients) versus 0.45 +/- 0.15 (five of 11 patients), P =.01].
CONCLUSION: Even locally advanced and metastatic sacrococcygeal GCT can be successfully treated with up-front cisplatinum-based chemotherapy followed by delayed but complete tumor resection.
Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study.Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ, Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F, Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP.
St Jude Children's Research Hospital, Memphis, TN, USA.
J Clin Oncol 1999 Jul;17(7):2137-43 Abstract quote PURPOSE: To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%.
PATIENTS AND METHODS: Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death.
RESULTS: Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy.
CONCLUSION: Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.
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