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Table 2 Treatment details for 9 patients with esthesioneuroblastoma

From: Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees

Pt.

Protocol type

Initial

surgery

CT (No. of cycles)

Response to CT

Delayed

surgery

RT

(dose)

Outcome (years after diagnosis)

Long-term sequelae

1

RMS

Macroscopic residuals

VAdIA (9)

PR

No

No

DOD (0.7)

-

2

RMS

Biopsy

VAdIA (12)

CR

No

Yes

(53 Gy)

NED (11.5)

GH deficit, hypogonadism, hypothyroidism, chronic sinusitis, hypovision and cataract, hearing loss, dental abnormalities, facial bones hypoplasia

3

RMS

Biopsy

VAdIA + Carbo/E (5)

PR

Microscopic residuals

Yes

(50 Gy)

NED (13)

Palate deformity

4

RMS

Biopsy

VAdIA (12)

MR

Complete resection

Yes

(60 Gy)

NED (14)

Hypothyroidism, xerostomia, oligospermia

5

RMS

Microscopic residuals

VAdIA (9)

NE

No

Yes*

(42 Gy)

NED (9.2)

Chronic headache, hypothyroidism, attention-deficit/hyperactivity disorder

6

NBL

Macroscopic residuals

VAdC + CDDP/E (6)

SD

Microscopic residuals

Yes

(48 Gy)

NED (11.1)

loss of sense of smell, facial bone hypoplasia, recurrent keratoconjunctivitis, maculopathy

7

NBL

Biopsy

VAdCA + CDDP/E+

i.t. MTX (15)

PR

No

Yes

(60 Gy)

NED (23)

Amaurosis, hypothyroidism, GH deficiency, xerostomia, facial bones hypoplasia

8

NBL

Lymph node biopsy

VAdC/CDDP (7)

CR

No

Yes

(47 Gy)

NED (20.2)

Peripheral neuropathy

9

RMS

Macroscopic residuals

VAdC (12)

NE

No

Yes

(60 Gy)

NED (17.1)

Chronic sinusitis

  1. Pt: patient; CT: chemotherapy; RT: radiotherapy; RMS: rhabdomyosarcoma; V: vincristine, Ad:adriamycin, I: ifosfamide, A actinomycin-D; PR: partial response; DOD: dead of disease; CR: complete response; Gy: grays; NED: not evidence of disease; GH: growth hormone; Carbo: carboplatin; E: etoposide; PR: partial response; MR: minor response; NE: not evaluable; NBL: neuroblastoma; C: cyclophosphamide; CDDP: cisplatin; SD: stable disease; i.t. MTX: intrathecal methotrexate