Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHADNET) Syndrome: A Systematic Review

Table 6

Tumor presentation of patients with ROHHADNET syndrome (IPD and aggregate data).

Author, year [ref]	Type/histology	Location/size	Associated symptoms/signs	Treatment
Park, 2010 [7]	Ganglioneuroma	Right adrenal	N/A	IVIG
Gordon, 2015 [13]	Ganglioneuroblastoma	Left adrenal	N/A	N/A
Tellingen, 2015 [14]	Ganglioneuroma	N/A	N/A	N/A
Grudnikoff, 2013 [15]	Ganglioneuroma	N/A	N/A	resection
Patwari, 2011 [16]	Ganglioneuroblastoma	Right paraspinal	N/A	N/A
Bougnères, 2008 [19]	Ganglioneuroma (6 patients)	1 mediastinal; 2 right adrenal; 3 left adrenal	N/A	N/A
Paz-Priel, 2011 [20]	Ganglioneuroblastoma	Retroperitoneal mass	Opsoclonus- myoclonus-ataxia syndrome	resection, cyclophosphamide, IVIG
Chandrakantan, 2012 [21]	Ganglioneuroblastoma	Left adrenal	N/A	resection
Sumanasena, 2012 [23]	Ganglioneuroma	Left adrenal	N/A	resection
Abaci, 2013 [3]	Ganglioneuroma/ intermixed type with favorable histology	Retroperitoneal mass; (6.5 × 3.5 × 2.0 cm)	N/A	Resection, cyclophosphamide, IVIG, dexamethasone
Atapattu, 2015 [24]	Ganglioneuroma	Right adrenal	N/A	resection
Ucar, 2013 [8]	Hamartomatous mass with neural elements of benign nature	Parahilar mass (2.5 cm)	N/A	Resection
Sethi, 2014 [25]	Ganglioneuroblastoma	Right adrenal mass; (4.0 × 3.0 × 4.0 cm)	N/A	N/A
Baronio, 2013 [27]	Ganglioneuroblastoma intermixed	N/A	Hypertension, Cushing syndrome	Resection
Maksoud, 2015 [33]	Ganglioneuroma	Paravertebral mass (8.0 × 3.5cm) compressing the right ureter	Right hydroureteronephrosis	Resection
Sanklecha, 2016 [34]	Ganglioneuroblastoma	Paravertebral mass	Gait disturbance	Resection, chemotherapy (not specified)
Aljabban, 2016 [37]	Ganglioneuroma	Posterior mediastinal mass; (10 × 10 cm)	N/A	resection
Bagheri, 2017 [4]	Ganglioneuroblastoma	Mediastinal mass (1.5 cm)	N/A	N/A
Jacobson, 2016 [39]	Ganglioneuroma	N/A	N/A	resection

IVIG, intravenous immunoglobulin; N/A, not available for information.