Table 1. Demographic and clinical characteristics for the six patients presenting with esthesioneuroblastoma that was deemed unlikely to be resected with negative margins prior to induction therapy.
| Patient number | Age (y) | Gender | Kadish stage | Hyams grade | IT regimen (number of cycles) | Response to IT | Radiation before surgery | Surgical approach | Time from start of IT to surgery | Completeness of resection | Postsurgery adjuvant therapy | Disease recurrence | Survival (RFS/OS a ) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 44 | Male | C | N/A |
CAV,
2
EP,
2
EP (2 b ) |
PR | 6,480 cGy | Cranio-endoscopic | 7 months | Gross total, (-) microscopic margins | None | L, DM | DOD (29/79) |
| 2 | 22 | Male | D | High | CAV, 6 CAV, 2 CEP 1 | PR, SD c | 6,000 cGy | Bifrontal craniotomy | 12 months | Gross total, (-) microscopic margins |
Chemoradiotherapy to neck
d
(P b ; 6,000 cGy) |
None | AND (26/26) |
| 3 | 25 | Male | C | High | EP 3 | PR | None | Cranio-endoscopic | 3 months | Gross total, (-) microscopic margins | Chemoradiotherapy to primary tumor site (EP b ; 6,000 cGy) |
None | AND (28/28) |
| 4 | 51 | Female | D | N/A | EP, 5 P (2 b ) | SD | 4,500 cGy | Bifrontal craniotomy | 12 months | Gross total, (+) microscopic margins | None | R (delayed LN metastasis in unirradiated neck) | AWD (43/60) |
| 5 | 40 | Female | C | Inter-mediate | CEP 2 | SD | None | Bifrontal craniotomy | 2 months | Gross total, (-) microscopic margins | Radiotherapy to primary tumor site (5,580 cGy) |
L, R (delayed LN metastasis in unirradiated neck) | DOD (33/165) |
| 6 | 53 | Male | D | Low | P (2 b ), EP 3 | CR | 6,020 cGy | N/A | N/A | N/A | N/A | DM | DOD (11/11) |
Abbreviations: AND, alive, no evidence of disease; AWD, alive, with evidence of disease; CAV, cyclophosphamide, anthracycline, vincristine; CEP, cyclophosphamide, etoposide, cisplatin; cGy, centiGray; CR, complete response; DM, distant metastases; DOD, dead of disease; EP, etoposide, cisplatin; IT, induction therapy; L, local recurrence; LN, lymph node; N/A, not applicable; OS, overall survival; P, cisplatin; PR, partial response; R, regional recurrence; RFS, recurrence-free survival; SD, stable disease.
RFS and overall survival are shown in months from the time of the start of induction therapy.
Chemotherapy given concurrently with radiotherapy.
Patient 2 received six cycles of CAV followed by 6,000 cGy of external beam radiotherapy and achieved a PR but then had local progression very soon thereafter. He received two more cycles of CAV, then one cycle of CEP, and had stable disease before being taken to definitive surgery.
Patient 2 was treated with 6,000 cGy of adjuvant radiotherapy to right side of neck after surgery because one cervical lymph node that was surgically removed contained esthesioneuroblastoma. The primary tumor site was not irradiated a second time.