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Congenital Pyriform Aperture Stenosis
Joseph Junewick, MD FACR
over 7 years ago
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Case History


14 year old female with right lower quandrant pain; rule out appendicitis.


Case Detail

Anatomy: Genitourinary
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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Reveal Clinical Notes

Pathology – Ganglioneuroma with occasional neuroblasts.

Reveal Findings

US – Lobulated uniformly isoechoic left retroperitoneal mass.

CT – Noncalcified left paraaortic mass displacing the renal hilum anteriorly.

Reveal Discussion

Neuroblastoma is the most common extracranial solid neoplasm in childhood. Considered one of the “small round blue cell” tumors, it arises from primitive pleuripotent sympathetic cells in neural crests. The three classic histopathologic forms in increasing maturation and differentiation are neuroblastoma, ganglioneuroblastoma, and ganglioneuroma.

Neuroblastoma has an incidence of 10.5 cases per million per year. The average age at diagnosis is 22 months with 36% occurring before 1 year of age, 79% occurring before 4 years of age and 97% occurring before 10 years of age. The etiology of neuroblastoma is unknown but numerous environmental exposures and germinal mutation have been implicated. Neuroblastoma occasionally undergoes spontaneous differentiation and maturation.

Neuroblastoma can arise anywhere along the sympathetic chain. The abdomen (fairly evenly divided between the adrenal gland and the remainder of the abdominal sympathetic chain) is by far the most common location, accounting for 2/3 of cases. The paraspinal (thoracic more than cervical), pelvic and other regions account for remainder in decreasing order. Regional lymph node metastasis occurs in 35% of apparently localized tumors. Lymph node metastasis outside of the body cavity of origin represents disseminated disease. Hematogenous metastases are most common to bone marrow, bone, liver, and skin and infrequently to lung and brain. The incidence of metastatic disease increases with age; disseminated disease occurs in 25% under the age of 1 year and 68% over the age of 1 year.

Reveal Diagnosis

Ganglioneuroma

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