Dermatology Online Journal
Rare presentation of a congenital neuroblastoma
- Author(s): D'souza, Ignatius Edwin
- Patidar, Ramesh Chandra
- Mani, Ravichandran
- Shamseldeen, Mahmoud
- Jose, Joyce
- et al.
Rare presentation of a congenital neuroblastoma1. Department of Pediatrics, Gulf Medical College Hospital, Ajman, UAE
Ignatius Edwin D'souza MD MRCPCH1, Ramesh Chandra Patidar MD1, Ravichandran Mani MD1, Mahmoud Shamseldeen MS MD1, Joyce Jose MD2
Dermatology Online Journal 15 (11): 15
2. Department of Pathology, Gulf Medical University, Ajman, UAE
A term neonate born by caesarian section was found to have multiple shiny, pinkish purple, fleshy nodules all over the body in addition to the bluish subcutaneous nodules with massive hepatomegaly. Computerized tomography of the chest revealed a posterior mediastinal mass at the right paravertebral region; abdominal CT showed multiple hypodense lesions in the liver. A biopsy from one of these skin nodules confirmed the diagnosis of neuroblastoma. Multiple skin nodules are a rare presentation of congenital neuroblastoma.
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A male baby was born of a non-consanguineous marriage to a gestational diabetic mother with a birth weight of 3.3 kg. Antenatal ultrasound scan had revealed cysts in the kidneys and liver with a possibility of polycystic kidney disease. There was no family history of any significant illness. On examination this neonate had multiple shiny, pinkish purple, fleshy nodules on the skin that were firm in consistency with an erythematous base (Figs. 1 through 5). There were bluish subcutaneous nodules as well. He had hepatomegaly with a liver reaching the right iliac fossa and a lobulated, subcutaneous swelling near the right scapula measuring about 6 x 3 cm (Figs. 6 & 7).
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Complete blood count was normal. Ultrasound of theabdomen showed an enlarged liver with multiple variably sized hypoechoic cystic defects. Computerized tomography showed a posterior mediastinal mass at the right paravertebral region, measuring 25 x 24 x 15 mm in largest dimensions, with an enlarged liver containing multiple hypodense lesions. Multiple soft tissue density nodules were seen in the lung as well as in the skin and subcutaneous tissue. Urinary VMA was normal. Biopsy from one of the skin nodules clinched the diagnosis of neuroblastoma (Figs. 8, 9, & 10).
|Figure 8||Figure 9|
This neonate was put on a ventilator because of respiratory compromise. The child was given one dose of chemotherapy but developed renal failure. He progressively deteriorated and died on the tenth day of life. To our knowledge, multiple shiny, pinkish purple, fleshy nodules have not been described in congenital neuroblastoma, though subcutaneous nodules have been well described.
Similar firm cutaneous nodules have been reported in a term infant with neuroblastoma . An 8-week-old girl presented with firm, blue cutaneous nodules which blanched when rubbed and were surrounded with a halo of erythema . Congenital neuroblastoma has a good prognosis but 10-25 percent of these patients, especially neonates, are at high risk of death because of respiratory complications as a result of massive hepatomegaly .
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2. Lucky AW, McGuire J, Komp DM. Infantile neuroblastoma presenting with cutaneous blanching nodules. J Am Acad Dermatol 1982 Mar; 6(3): 389-91. [PubMed]
3. van Noesel MM, Hahlen K, Hakvoort-Cammel FG, Egeler RM. Neuroblastoma 4S: a heterogenous disease with variable risk factors and treatment strategies. Cancer 1997 Sep 1; 80 (5): 834-43. [PubMed]
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