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Lixana Vega Vega, MD Department of Surgery SUNY Downstate Medical Center

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Page 1: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

Lixana Vega Vega, MDDepartment of SurgerySUNY Downstate Medical Center

Page 2: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 3: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

•15 X13X10 cm complex cystic lesion posterior and anterior to sacrum.•No continuity with spinal canal

Page 4: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 5: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 6: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 7: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

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.

Page 8: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 9: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 10: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• Pt received abx postop. • POD #1 - Tolerated PO diet.• POD # 2 - + BM• POD # 3 – Discharge Home with PO abx, JP with

SS fluid.

Page 11: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 12: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

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

Page 13: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 14: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

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.

Page 15: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

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.

Page 16: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 17: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 18: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 19: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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 )

Page 20: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 21: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 22: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 23: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 24: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 25: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 26: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk
Page 27: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 28: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.

Page 29: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 30: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 31: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• Germ Cell Tumor• Tissues of 3 germ cell layers or

monodermal• Treatment – Complete resection• Excellent prognosis

Page 32: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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

Page 33: Lixana Vega Vega, MD Department of Surgery SUNY Downstate ... · • Germ cells are totipotential tumor types associated with the degree of cell differentiation. ... h iti it f lk

• 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.