Esthesioneuroblastoma Follow-up

Updated: Jan 02, 2018
  • Author: Michael Somenek, MD; Chief Editor: Herbert H Engelhard, III, MD, PhD, FACS, FAANS  more...
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Follow-up

Further Outpatient Care

Nasal irrigation with sterile isotonic sodium chloride solution begins within a few days after packing removal. Removal of nasal crusting should be performed regularly during initial postoperative visits. This problem is aggravated during radiotherapy but decreases in most patients after 1-2 years. It is a lifelong burden for which no good treatment or prevention is available.

Craniotomy sutures are removed 7-10 days after surgery. If a facial incision has been used, sutures are removed after 5 days

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Further Inpatient Care

In the first 48 hours following craniofacial resection, regular neurosurgical evaluation is required. ICU monitoring for at least 24 hours also is necessary. Duration of the lumbar drainage is controversial, varying from intraoperative only to as long as 7 days.

Aspiration of the nasal trumpets should be performed several times a day. Nasal packing is left in place for 5-7 days. Removal of large nasal packing is uncomfortable, and some pain medication could be administered before the procedure.

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Inpatient & Outpatient Medications

Antibiotic prophylaxis is usually started before surgery for esthesioneuroblastoma and includes coverage for gram-positive bacteria. Because good intracerebral penetration is preferred, ceftriaxone often is a good choice. Postoperatively, the authors continue antibiotics while the nasal packing remains in place.

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Complications

Craniofacial resection for esthesioneuroblastoma (ENB) has been associated with a complication rate of 10-15%. Higher rates of complications (up to 30%) have been observed in cases of revision or salvage surgery or surgery following radiation or chemoradiation therapy.

The most frequent problems are infections, including abscess surrounding the bone flap; meningitis; and, less often, subdural or epidural abscess.

Meningitis sometimes is related to the presence of a cerebrospinal fluid leak, because the closure of the anterior skull base is not always watertight.

Pneumocephalus has been another difficult problem to prevent. Although self-limiting in most cases, pneumocephalus can result in brain compression and requires needle aspiration through the trephine bony holes.

Blindness, secondary to optic nerve injury, and death either from intracerebral bleeding or internal carotid injury have become exceptional.

Inform patients that olfactory function is likely to be altered or absent after surgery. In addition, nasal crusting is a long-lasting problem that requires daily nasal irrigation for several years.

Delayed complications include necrosis of the frontal bone flap, lacrimal drainage obstruction, and frontal sinus mucocele.

Radiation-related complications also are rare and include lacrimal duct blockage and tearing, postradiation cataract, osteoradionecrosis of the frontal bone flap, radiation retinopathy, and blindness.

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Prognosis

See the list below:

  • Treatment results before the availability and use of modern diagnostic techniques were flawed, probably by the inclusion of cases of sinonasal undifferentiated carcinoma and sinonasal neuroendocrine carcinoma, 2 aggressive diseases associated with poor survival. The importance of correctly differentiating these cancers is highlighted in a recent report by Rosenthal et al. [40] For a discussion of posttreatment prognosis, see Kane et al. [9]

  • In recent series, the 5-year patient survival rates have varied from 50-80%, with the majority of large studies indicating patient survival rates of higher than 70%. In the 2001 meta-analysis by Dulguerov et al, the average 5-year survival was 45 ± 22% (range, 0-86%). [20]

  • Whether the Kadish classification system has a definitive prognostic value remains unknown, but the available survival data for stages A, B, and C are 72%, 59%, and 47%, respectively. According to the Dulguerov staging system, the survival rates were 81% for T1, 93% for T2, 59% for T3, and 48% for T4.

Prognostic survival factors that emerged in the meta-analysis by Dulguerov et al include lower Hyams histopathologic grade (56% for grades I-II vs. 25% for grades III-IV) and absence of neck lymph node metastasis (64% for N0 vs. 29% for N+). [20]

Other possible but unconfirmed prognostic factors include a low proliferative index, the completeness of the surgical resection, and tumor shrinkage after induction chemotherapy.

Survival data categorized according to treatment modality favored regimens that include surgery.

The most frequent recurrence is local, with rates from 20-40% (29% in the Dulguerov et al meta-analysis). Craniofacial resection followed by radiation seems to result in fewer recurrences, a rate of approximately 10%. Salvage after local recurrence is possible in one third to one half of cases.

Regional recurrence, while disease at the primary site remains under control, occurs in 15% of cases and is salvageable by further treatment in 25-50% of cases. Distant metastasis with locoregional control occurs infrequently (8%) and carries a dismal prognosis.

Recurrence may occur years after the completion of treatment, often more than 10 years; therefore, prolonged follow-up is required, although the optimal frequency of medical visits and the necessity of radiologic studies have not been precisely determined.

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