History:

There are three cases of congenital neoplasms presented here.

Example 1:

A fetus was stillborn at term. Ultrasonography had shown a large eccentrically located mass lesion in the region of the head.

This mass arises in the nasopharyngeal region. Sectioning the mass revealed areas of bluish-white cartilage and bone, cystic areas, and solid regions. Microscopically, a variety of tissue types are represented. Cartilage and bone are seen, along with primitive mesenchymal tissue.

Example 2:

A 19-year-old G2 P1 woman had ultrasonography at 19 weeks that demonstrated a mass lesion of the fetus' pelvic region, and an elective termination of pregnancy was performed.

Sections of the left lower extremity mass demonstrated spongy red tissue. Microscopically, numerous vascular spaces are present lined by thin endothelium and filled with RBC's and fibrin clots. Occasional dystrophic calcification is present.

Example 3:

A liveborn 36 week gestational age infant lived for only 36 hours and had severe fetal hydrops. Ultrasonography had revealed a mass lesion in the right abdominal region.

The mass on sectioning demonstrated areas of necrosis as well as calcification. The mass extended from liver to lower pelvis and obliterated the right kidney. Microscopically, small round blue cells with scant cytoplasm are present in this neoplasm.

Questions:

1. What are the diagnoses?

1. Example 1: Teratoma

2. Example 2: Hemangioma

3. Example 3: Neuroblastoma

2. What are the most common childhood malignant neoplasms? What are the most common benign childhood neoplasms?

Malignant: Leukemia/lymphoma, brain tumors, and soft tissue sarcomas are the most common. Other malignancies include Wilms tumor of kidney, bone tumors, and retinoblastoma of eye. Overall, these malignancies are still rare in this population. Though rare, childhood cancer is the leading cause of death for ages 1 - 15 (because other causes of death at this age are even less common). The incidence of congenital neuroblastoma is 1:8000.

Benign: About one third of all soft tissue neoplasms in the first year of life are hemangiomas or lymphangiomas. Fibromatoses are also common. Teratomas occur in midline regions (sacrococcygeal, cerebral, nasopharyngeal).

3. What are some factors that determine the prognosis?

Size and location are important, for even histologically benign neoplasms can obliterate normal tissues, be difficult to resect, or recur with incomplete resection. Malignant neoplasms have the capacity for invasion and metastases, but in childhood many malignant neoplasms remain localized (such as retinoblastoma). Some childhood malignancies have a good response to chemotherapy (such as some leukemias and lymphomas). Staging and grading of neoplasms is important to determine therapy and prognosis. Genetic markers and tumor karyotypes are also prognostic indicators.