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Malignant Germ Cell Neoplasm , Peadiatric Yolk Sac Tumor
01/26/2010
Case submitted by Dr. Rugvedita Parakh and Dr. Mahul Amin.

7 months male child presented with scrotal mass of 4.2 cm size and elevated AFP levels.
Sex:
male
History:
7 months male child presented with scrotal mass of 4.2 cm size and elevated AFP levels.
Note:
Clinical features

1. Physical examination -

Painless scrotal mass may be accompanied by hydrocele (10%), 3% with history of recent trauma

2. Serum markers -

Elevated AFP levels in an infant with yolk sac tumor of the testis must be interpreted with caution because of the normal physiologic elevation seen in infancy. Serial monitoring of the AFP degradation curve helps in identifying true residual disease in infants with an initial elevated AFP and yolk sac tumor

3. Imaging –

· Ultrasonography - The evaluation of boys with large or tense hydrocele to identify cystic components in a testicular lesion that would suggest a teratoma or epidermoid cyst rather than a yolk sac tumor.

· Color Doppler to detect intratesticular neoplasms

Histopathology

1. Gross- Well encapsulated or circumscribed tumors with friable, pale gray and mucoid appearance. Soft consistency and microcystic appearance on cross section are peculiar. They may show hemorrhagic and necrotic components.

2. Microscopy-

· Four different patterns are described which do not influences prognosis but may cause diagnostic challenges- (1) pseudopapillary, (2) reticular, (3) polyvesicular vitelline, and (4) solid. The pseudopapillary pattern is most commonly seen in children. The characteristic histopathologic finding of yolk sac tumor remains the Schiller-Duvall body- a papillary projection associated with perivascular sheets of cells.

· Stroma - cellular, spindle shaped, and reminiscent of smooth muscle appears to represent a chemoresistant, pluripotential cell population may give rise to a sarcoma (of spindle cell type against a background of a myxoid to collagenous stroma) after treatment

· Perivascular Schiller–Duval body: most distinctive feature of yolk sac tumor have been compared to structures seen in rat placenta and represent an attempt to form yolk sacs

· Hyaline intracytoplasmic and extracytoplasmic round inclusions

Cytological examination

· Richly cellular smears with a combination of morphological patterns.

· Tumor cells are most commonly arranged in papillary groups, tight cell clusters and form acinar structures.

· Large, moderately pleomorphic cells with hyperchromatic nuclei and moderate amount of cytoplasm, some displaying vacuolation, and may displace the nuclei eccentrically.

Immunohistochemistry

Most immunoreactivity for AFP:

Consistently positive for keratin, glypican-3 and GATA-4

Staging

Children's Cancer Group (CCG)/Pediatric Oncology Group (POG) intergroup study

Stage I Tumor is limited to testis. (No evidence of disease is present beyond the testis by clinical, histologic, or radiographic examination.)

Complete resection is possible by high inguinal orchiectomy.

An appropriate decline in serum tumor markers (AFP t½ = 5 days).

Patients with normal or unknown tumor markers at diagnosis must have a negative ipsilateral retroperitoneal lymph node sampling to confirm stage I disease.


Stage II Microscopic disease is located in the scrotum or high in the spermatic cord (<5 cm from the proximal end).

Retroperitoneal lymph node involvement is present (<2 cm).

Serum tumor markers are persistently elevated.

Transcrotal orchiectomy is possible.
Stage III Retroperitoneal lymph node involvement (>2 cm) is present.

There is no visible evidence of visceral or extra-abdominal involvement.
Stage IV Distant metastases are present, including the liver.
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