Ganglioneuroblastoma: Unusual presentation as a pleural mass mimicking mesothelioma

Our plain-English summary of Ganglioneuroblastoma: Unusual presentation as a pleural mass mimicking mesothelioma, by B.B. Jain, S. Ghosh, M.M. Das, et al (Lung India, 2016).

The study

• Authors: B.B. Jain, S. Ghosh, M.M. Das, S. Chattopadhyay
• Journal: Lung India
• Published: 2016;33(2):199-201
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Ganglioneuroblastoma (GNB) is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system, and usually occurs in young children. We present a case of GNB occurring as pleural mass in a 2-year-old boy, which led to diagnostic confusion. On fine-needle aspiration cytology (FNAC), it was misinterpreted as mesothelioma. He underwent thoracotomy with excision of the mass. Histopathological findings showed features of a biphasic tumor suggestive of mesothelioma. Immunohistochemistry (IHC) performed for mesothelioma markers were inconclusive. On review of the histology slides, GNB was considered, which was subsequently proven by IHC. The rarity of this tumor, along with its nearly restricted occurrence at a young age, necessitates a strong suspicion in patients presenting with a symptomatic intrathoracic mass.

In plain English

This article is a case report by pathologists at Medical College, Kolkata, India. It describes one 2-year-old boy who came to doctors with fever and breathing difficulty. A chest X-ray and CT scan showed a large solid mass attached to the pleura (the lining around the lung) on the right side. A needle sample of the mass was read as a tumor of mesothelial cells, possibly mesothelioma. The mass, measuring 8 by 6 by 5 cm, was removed by surgery. The first tissue diagnosis was malignant mesothelioma of biphasic type, but stains for mesothelioma markers were inconclusive. When the slides were reviewed again, the tumor was found to be a ganglioneuroblastoma, a rare tumor of developing nerve cells seen almost only in children. Chromogranin and S100 stains confirmed it. At three months a CT scan showed ill-defined pleural thickening near the original tumor site. A bone scan and bone marrow tests were unremarkable, and the boy was in good clinical health at six months. The authors concluded this tumor should be considered in children with a chest mass. The article does not discuss asbestos.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 2-year-old boy with fever and respiratory distress; authors from the Department of Pathology, Medical College, Kolkata, West Bengal, India
• Main results:
  • CT of the thorax showed a large, solid, pleural-based, noncalcified mass with partial collapse of the right lower lobe; no communication with the spinal canal
  • Fine-needle aspiration cytology was interpreted as a tumor of mesothelial cell origin, possibly mesothelioma
  • The excised mass measured 8 × 6 × 5 cm, and the initial histological diagnosis was malignant mesothelioma of biphasic type
  • Immunohistochemistry for mesothelioma markers (calretinin, cytokeratin, EMA, WT1, vimentin) was inconclusive for mesothelioma
  • On slide review ganglioneuroblastoma was suspected; repeat immunohistochemistry showed chromogranin positivity in ganglion cells and diffuse S100 positivity in background spindle cells, giving a final diagnosis of GNB intermixed (Schwannian stroma-rich)
  • Follow-up: urinary catecholamines were normal; CT at 3 months showed ill-defined thickening in the posterior parietal pleura near the initial tumor bed; bone scintigraphy and bone marrow were unremarkable; the patient was clinically in good health at 6 months
  • The authors stated they found no prior literature of this tumor presenting as a pleural mass
• The authors' conclusion: The authors concluded that ganglioneuroblastoma should remain one of the differential diagnoses in children presenting with an intrathoracic mass, given its rarity and atypical presentations

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Fine-Needle Aspiration, Cytology, Thoracotomy, Biphasic Mesothelioma, Immunohistochemistry, Histology, Intrathoracic.

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