lixana vega vega, md department of surgery suny downstate ... · • germ cells are totipotential...
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Lixana Vega Vega, MDDepartment of SurgerySUNY Downstate Medical Center
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• 10 y/o female presented with mass at lower back.
• Mass noticed 5 years ago, but enlarged over the last 2 years.
• Denied lower back or abdominal pain.• PMH
• Born full term without complications. • PE
• Well developed 10 y/o female, 146 lbs.• 10 x 12 cm mass over sacrum.• Abdomen- soft, nontender, nondistended.• DRE- mass not palpable• No LE motor or sensory deficit.
• Labs• CBC, Chem, Coags - WNL
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•15 X13X10 cm complex cystic lesion posterior and anterior to sacrum.•No continuity with spinal canal
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Posterior Aproach• Ampicillin, Gentamycin,
Flagyl given. • Prone position• Semilunar incision• Mass attached to periostium
of sacrum and coccyx.• Coccygectomy• Yellow fluid from cystic
cavity• Dissection in plane
posterior to rectum.• JP drain over muscle
closure.
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• Multiloculated cystic mass 24.5 x 14 x 8 cm • External component• Internal component
• Culture of yellow thick fluid – negative.
• Epidermoid cyst with chronic inflammation.
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• Pt received abx postop. • POD #1 - Tolerated PO diet.• POD # 2 - + BM• POD # 3 – Discharge Home with PO abx, JP with
SS fluid.
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Teratoma Other histologic types
Seminoma (Dysgerminoma)Yolk sac tumor (Endodermal
Sinus)Embryonal Carcinoma
PolyembryomaChoriocarcinoma
Mixed GCT
Gonadal Extragonadal
Ovarian Testicular
SacrococcygealHead and Neck
MediastinalRetroperitoneal
rare
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• 2.4 cases per million children
• 1% of cancers diagnosed in children younger than 15 years
• Gonadal and Extragonadal sites
• Originate from the primordial germ cell.
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Migration of Germ cells – 4th and 5th Weeks Gestation • Germ cells migrate from yolk sac to the gonadal ridges and
incorporate into gonadal tissue.
• Migration mediated by the c-kit receptor and stem cell factor.
• Failure in migration deposition of germ cells in abnormal locations:• Anywhere from the brain to the coccygeal area, usually in the midline.
• Sacrococcygeal area, mediastinum and retroperitoneum.
• Malignant transformation can occur at any of these sites.
• Germ cells are totipotential tumor types associated with the degree of cell differentiation.
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Staging for Malignant ExtragonadalGerm Cell Tumors
Stage I: Localized disease, with complete resection at any site (coccygectomy for sacrococcygeal site); negative tumor margins; tumor markers positive or negative
Stage II: Microscopic residual disease; capsular invasion; negative lymph nodes or microscopic lymph node involvement; tumor markers positive or negative
Stage III: Gross residual disease; gross lymph node involvement; cytologicevidence of tumor cells in ascites or pleural fluid; tumor markers positive or negative
Stage IV: Distant metastases involving lungs, liver, brain, bone, distant nodes, or other sites.
*Children's Oncology Group and the National Cancer Institute.
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• Most frequently occurring GCT
• Extragonadal infancy and early childhood
• Gonadal older children • More than half of teratomas
are observed at birth.• Diagnosed generally during
the first year of life.• 20% - malignant component
• endodermal sinus tumor.
Site of Tumor Occurrence
• From Shamberger RC: Teratomas and germ cell tumors. In O’Neill JA Jr, Grosfeld JL, Fonkalsrud EW, et al (eds): Principles of Pediatric Surgery, 2nd ed. St Louis, Mosby, 2004.
Site Incidence (%)Sacrococcygeal region
45-65
Anterior mediastinum
10-12
Gonadal (ovary and testis)
10-35
Retroperitoneum
3-5
Cervical area 3-6Presacral area 3-5Central nervous system
2-4
Other rare sites (e.g., liver, kidney, vagina, stomach)
<1
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• Solid, cystic, or mixed lesions. • Tissues from each of the three germ layers.
• Ectoderm• Epidermal and dermal structures – hair, sebaceous and
sweat glands, teeth. • Neuroepithelial and glial tissue.
• Mesoderm• Fat, cartilage, bone, and muscle.
• Endoderm• Intestinal epithelium, Pancreatic, adrenal, and thyroid
tissue.
• One germ cell layer may predominateMonodermal
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• Most common Teratoma• Most common neoplasm of the fetus and newborn• Incidence of 1 in 20,000 to 40,000 live births• Female predominance from 2:1 to 4:1 • 17% have malignant histologic or clinical features.• 10% of patients have a family history of twinning.• Coexisting anomalies in10-20%
• Imperforate anus, anorectal stenosis, spina bifida, genitourinary malformations, meningomyelocele, and anencephaly.
• Structural abnormalities of juxtaposed organs from displacement by the teratoma.
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• Type I (46.7%)• external with minimal presacral
extension. • Type II (34.7%)
• external with significant intrapelvic portion.
• Type III (8.8%) • primarily pelvic and abdominal,
but apparent externally. • Type IV (9.8%)
• presacral, no external manifestation.
Altman et al. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey—1973. J Pediatr Surg1974;9:389 )
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• Malignancy correlates with • Anatomic type
• 8% in type I versus 38% in type IV• Age at diagnosis
• 8% malignant dx at birth• 40-80% malignant dx older than 6
mo • Male gender
• Solid tumor > cystic• No correlation with size of the
tumor.
• Malignant tissue• Yolk sac tumor or embryonal
carcinoma.
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• Diagnosed with Ultrasound - 22 and 34 weeks' gestation. • Significantly less favorable prognosis than does diagnosis at
birth.• Mortality rate for SCT diagnosed in neonates is 5% vs that for
fetal SCT is close to 50%. • Hydrops or polyhydramnios and placentomegaly associated
with fatal outcome. • US findings
• Mass arising from the sacral area of the fetus • Mass composed of solid and cystic areas, foci of calcification.
• Most prenatally diagnosed SCTs are extremely vascular and can be seen on color-flow Doppler.
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• Physical examination• 90% of SCTs are noticed at delivery. • Protruding mass extending from the coccygeal region. • DRE to assess intrapelvic component
• Intrapelvic tumors• Delayed postnatal presentation
• Infants and children between 4 months and 4 years of age. • Clinical presentation
• Symptoms from tumor compressing the bladder or rectum, constipation, anal stenosis.
• Sacral defects and anorectal malformations
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• Imaging studies • PXR
• Sacral defects or tumor calcifications.• CT scan with IV and rectal contrast defines the
• Intrapelvic extent of the tumor• Lymph node or distant metastases (liver) • Urinary tract displacement or obstruction
• MRI • When spinal involvement is suspected or if the diagnosis is in
doubt.
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• Lumbosacral myelomeningocele• Similar findings on US.
• Assessment of the integrity of the fetal spine - MRI• Assessment of lower extremity function.
• Other DDx• Neuroblastoma• Hemangioma• Leiomyoma• Lipoma
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• The treatment of choice is complete surgical resection on an elective basis early in the newborn period. • Emergent surgery for tumor rupture or hemorrhage.
• Operative approach• Tumors with significant external component (type I and
II)• Posterior sacral approach
• Tumors with extensive intrapelvic component (type III and IV)• Abdominal approach
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• Operative goals • Complete tumor resection • Resection of the coccyx to prevent tumor recurrence
• Recurrence rate as high as 37% if coccyx is not removed.• Reconstruction of the muscles of anorectal continence • Restoration of a normal perineal and gluteal appearance.
• Mortality - hemorrhagic shock. • Morbidity
• intraoperative hemorrhage• fecal incontinence
• 30% to 40% of premature infants, large SCTs, levator muscles are severely attenuated.
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• Indicated when malignant component is identified. • Platinum-based chemotx (cisplatin) most successful• Other agents- etoposide, bleomycin. • Survival rates -88% with local disease and 75% with distant
metastases.
• For unresectable primary malignant tumor, chemotherapy decreases size of tumor facilitating subsequent resection.
• Localized malignant recurrence• Complete resection with adjuvant chemotherapy.• Radiation tx rarely given
• Metastatic foci in the lungs and liver.
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• The long-term outcome in newborns with SCT is generally excellent.
• Age at diagnosis - dominant prognostic factor. • Diagnosis made before 2 months of age or excision is performed before 4
months of age, the malignancy rate is 5% to 10%.• Cystic tumor (mature) better prognosis. • Solid tumors (immature) hemorrhage, malignancy.• The Pediatric Onoclogy Group–Children's Cancer Group study
(Marina et al, 1999)• 80% event-free survival and 100% survival with complete resection alone
in immature teratomas, even when microscopic foci of yolk sac tumor were present.
• Children's Cancer Group study (Rescorla et al, 1998)• 11% tumor recurrence rate with mature teratoma and a 4% recurrence rate
with immature teratoma. • Although 43% to 50% of these recurrences were malignant, the
h iti it f lk ( d d l i ) t lt d i hi h
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• Close follow-up at 3-month intervals for 3 to 4 years is recommended.
• Physical exam• DRE- may detect a presacral recurrence
• Imaging if suspicious for local recurrence• Abdominal US• Abdominal CT scan or MRI
• Chest and Abdomen CT scan• R/o metastasis
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• Germ Cell Tumor• Tissues of 3 germ cell layers or
monodermal• Treatment – Complete resection• Excellent prognosis
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• Grosfeld: Pediatric Surgery, 6th ed. Teratomas and other Germ Cell Tumors.
• Holcomb & Murphy: Ashcraft's Pediatric Surgery, 5th ed. Teratomas, dermoids and other soft tissue tumors.
• Young Jr JL, Ries LG, Silverberg E, et al: Cancer incidence, survival and mortality for children younger than age 15 years. Cancer 1986; 58(2 Suppl):598
• Altman RP, Randolph JG, Lilly JR: Sacrococcygealteratoma: American Academy of Pediatrics Surgical Section Survey 1973. J Pediatr Surg 1974; 9:389.
• Cushing B, Perlman E, Marina N, et al: Germ cell tumors. In: Pizzo PA, Poplock DG, ed. Principles and Practice of Pediatric Oncology, 2nd ed. Philadelphia: JB Lippincott; 1993.ch 36
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• Gross RE, Clatworthy HW, Meeker IA: Sacrococcygealteratoma in infants and children. Surg GynecolObstet 1951; 92:341.
• Marina NM, Cushing B, Giller R, et al: 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. J Clin Oncol 1999; 17:2137.
• Rescorla FJ, Sawin RS, Coran AG, et al: Long-term outcome for infants and children with sacrococcygealteratoma: A report from the Children's Cancer Group. J Pediatr Surg 1998; 33:171.