Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

Table 1

Reported cases of children and adolescents with intrathoracic MPNST in the English-language literature.

Reference	Age (years)	Sex	Symptoms	Location	Metastasis	NF1	Operation	Chemotherapy	Radiotherapy (cGy)	Outcome
Elli et al., 2007 [16]	13^1	Male	LUQ pain, weight loss	Lt paravertebral	—	Yes	Total resection	VICE, Ep, and A	Local, 4680	DOD, 14 months
Komori et al., 2003 [17]	12	Female	Chest pain, dyspnea	Rt paravertebral	N/A	No	Partial resection	N/A	N/A	DOD, 15 months
Muwakkit et al., 2006 [18]	2.5	Male	Malaise, fatigue, anorexia, and palpitations upon exertion	Rt pulmonary	N/A	No	Total resection and lobectomy	VDC	Not done	N/A
Imazu et al., 2006 [19]	12	Female	Neck swelling^2	Rt paraclavicular	—	Yes	Total resection	Not done	Not done	NED, 12 months
Moharir et al., 2010 [20]	8	Male	Weight loss, respiratory symptoms	Lt hemithorax	—	No	Subtotal resection	Done	Not done	DOD, 2.5 months
Moharir et al., 2010 [20]	12	Female	None (incidental)	Rt paraspinal	—	Yes	Total resection	Done	Not done	NED, 2 years
Present case	16	Female	Chest pain, respiratory problem	Rt anterior	Lung	No	Total resection	VDC/IE	Rt lung and pleura, 6600	DOD, 9 months

1His tumor was diagnosed with MPNST associated with some angiosarcoma components.
2 Her tumor was located at cervicothoracic lesion.
A: actinomycin-D; C: cyclophosphamide; D: doxorubicin; DOD: died of disease; E: etoposide; Ep: epirubicin; I: ifosfamide; Lt: left; LUQ: left upper quadrant; N/A: not assessed; NED: no evidence of disease; NF1: diagnosis of neurofibromatosis type 1; Rt: right; V: vincristine.