| Case Presention
Fetal Supra-Renal Mass—Diagnosis and Implications
Dr. Hema Divakar Consultant Obsterician
Dr. Chitra Ganesh Consultant Sonologist
Dr. Ramesh Consultant Paediatric Surgeon
Mrs. Bini Vinod, 29yrs old GэPıAı with an uneventful antenatal period was referred for a mid pregnancy scan. Biometry, Structural survey, Liqour and placental position were reported normal.
Repeat scan at 38 ½ weeks for assessment of Fetal weight & AFI detected the following findings.
Fetal left renal region showed a well defined Ovoid, solid and cystic space occupying lesion measuring 68mm x 48mm x 52 mm. Fetal kidneys, bladder and liquor were normal & there were no other anomalies.
Differential diagnosis of neuroblastoma / teratoma / extralobar pulmonary sequestration was considered.
After Vaginal delivery of a 3.75 kg female with good apgars, a Paediatric surgeon was called to evaluate the case.

Irregular, ballotable mass was palpable in the left lumbar region.
CT Scan of the abdomen showed a large 80mm x 70mm x 80mm soft tissue mass with calcifications in the left suprarenal fossa diagnosed as Neuroblastoma .Mass extended across the midline anterior to aorta.

Left kidney was displaced into iliac fossa , appeared normal, with no involvement.
α FP, HCG, VMA were assessed to rule out involvement of adrenal glands. VMA & HVA are known to be increased in adrenal cystic masses secondary to hemorrhage.
Laprotomy with excision of tumor was planned on Dз and the mass along with left kidney had to be removed, as the renal blood vessels were traversing the mass.

Gross Appearance of the globular mass measuring 80mm x 65mm x 50mm was firm to cystic with capsule and cut section revealed brownish yellowish cystic areas with cartilage and bone. Soft areas showed jelly like material.
Microscopic Examination showed immature neuro epithelial element & glial tissue (> 75%). Adrenal gland was embedded into this tissue. Papillary structures, glial tissue, internal glands, Cartilage, bone, fat, muscle and sweat glands ducts were also seen.

Final Impression of immature teratoma-(retroperitoneal) was made... Aggressive treatment with surgical removal present excellent outcomes as in the present case. Because this mass was large, Observation for spontaneous regression was not favoured

Conclusions Prenatal diagnosis of fetal intra abdominal masses is fairly easy & does cause an overwhelming anxiety to the Sonologist, Parents & the Obstetrician. Fortunately, majority of these cystic & solid masses are treatable and recurrent risk is almost unknown.
If there are no associated anomalies or hydrops, the survival rate after surgical intervention is very good. Prenatal diagnosis offers an opportunity for early intervention with excellent prognosis.
Additional Information:
Neuroblastoma:
Synonyms: Ganglioneuroblastoma, neuroblastoma in situ. BDE: Cancer, neuroblastoma.
Definition: Malignant neoplasm of poorly differentiated nerve cells of embryonic type.
Prevalance: 0.3-1:10,000. M1: F1
Neuroblastoma is the most common malignant tumor in infancy and early childhood, originating anywhere along the sympathetic nervous system, and in the adrenal gland. More than half of neuroblastomas are in the abdomen, and two- thirds of these originate in an adrenal gland. Fifteen percent of neuroblastomas are thoracic, arising posteriorly along the sympathetic chain. Other sites include the cervical region, Sympathetic chain in the abdomen, nasopharynx and brain.
More than half of patients are less than two years of age at diagnosis. Seventy – five percent of tumors are discovered prior to four years of age. The prognosis ranges from over 90% survival if discovered in patients under one year of age, to less than 10% survival when discovered in older children. Accurately early diagnosis is crucial, as the best prognosis is in the youngest patients.
Pathogenesis: Defect in neuroblast maturation with embryonal sympathetic ganglion cells undergo malignant transformation. Associated with n-mycogene and tumor cell ploidy: if n-mycogene is positive and cells are diploid, then the prognosis is worse.
Diagnosis: Suprarenal or posteromediastinal mass with over 95% of children having elevated catecholamine metabolites. Values in fetuses are not known. Occasionally the mother may exhibit signs due to the increased catecholamine.
Metastatic sites: Frequent: Bone lumph nodes, bone marrow,liver, skin;
occasional: extradural extension into spinal canal, rare: lung.
Associated anomalies: Hydrops, neurofibromatosis, Hirschprung”s disease, some chromosomal abnormalities. May be part of the fetal hydantoin and fetal alcohol syndromes.
Differential diagnosis: Adrenal site: renal duplex anomaly or tumor, adrenal cyst or hemorrhage is less likely.
Extra-adrenal site: Solid mass of lung,
Teratoma:
Germ cell tumor – drives from isolation of one or more blastomeres, fertilization of a polar body or parthogenetic development of egg.
The most extreme form is Fetus in fetu – the most extreme form of this type of tumor – embryonic duplication – abdominal, scrotal or intra cranial mass with an axial skeleton.
Teratoma have tissues from each of 3 layers of Embryonic disc with the ectodermal components, especially brain tissue – most represented. Mesodermal derivatives- fat / cartilage bone/ muscle.
The most common is Sacrococcygeal – 1: 35-40,000 is 4 times as frequent in female than in male fetus. These grow rapidly to large dimensions only a small % are malignant
NIHF is secondary to high output failure in fetuses with teratomas.
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