pediatric sinonasal non-ewing’s sarcoma: case report and ...with extra-sinonasal involvement....

ALBANY MEDICAL

COLLEGE

ALBANY MEDICAL

CENTER

OBJECTIVE

BACKGROUND

CASE REPORT

DISCUSSION

IMAGES

This is a rare care of non-Ewing’s pediatric

sinonasal sarcoma fully resected through

endoscopic endonasal approach. Patient age and

clinical stage of malignancy at initial finding are

both correlated with long-term post-diagnosis

survival rates3.

The patient in this case was young (10 years

old), his tumor was diagnosed early and it was low

grade. Thus, chemotherapy or radiation was not

required and the patient has a good prognosis.

Though the patient did develop left frontal

sinusitis and required a sinusotomy one year after

tumor excision, nasal function was eventually

restored. The patient is currently free of disease,

27 months after surgery.

To present a rare case of sinonasal non-

Ewing’s sarcoma in a pediatric patient. Literature

review of sinonasal sarcoma is provided.

CONCLUSIONS

A 10-year-old boy with a history of

progressively worsening nasal obstruction and

congestion presented with unilateral epistaxis, pain

in the nose and maxillary sinuses, and pressure in

the left eye. Nasal endoscopy showed a large mass

in the left nasal cavity extending toward the

maxillary sinus and nasopharynx. Pre-operative

MRI (Fig. 1) showed an extensive lesion in the left

ethmoidal sinus area. Intra-operative images

showed a large polypoid mass extending to the

maxillary sinus, sphenoid sinus, and partial left

frontal sinus. Erosion of the left lamina papyracea

was also present.

Complete resection of the tumor was achieved

with preservation of the anterior cranial base bone

since the mucosa of the ethmoidal roof was not

involved (Fig. 2). Post-operatively, the patient

noted remarkable improvement of nasal breathing

and normal sleep, despite some minor epistaxis.

No adjuvant therapy was recommended at our

tumor board.

Sarcomas of the sinuses or nasal cavities are

extremely rare in the pediatric population.

Tumors in general of the head and neck region are

not common in the pediatric population; only

about 10% of all pediatric malignancies occur in

this location. Of those tumors, even fewer (14%)

are malignancies of non-epithelial or connective

tissue, and can be characterized as sarcomas1.

Most sarcomas involving the nasopharynx or

paranasal sinuses tend to be large compared to

other types of tumors of the head and neck, and

are also associated with considerable destruction

of bone. Patients present with symptoms that are

often nondescript and similar to a common cold2.

Symptoms can vary from nasal obstruction and

nosebleeds to facial deformities, ocular and

neurological symptoms in case of large tumors

with extra-sinonasal involvement.

Here, we present a report of a sinonasal non-

Ewing’s sarcoma in a pediatric patient who

presented with facial pain and nasal obstruction.

The tumor was initially thought to be an

antrochoanal polyp. Complete tumor excision via

endonasal endoscopic approach was performed

and outcome was monitored by frequent follow-

ups.

Figure 1. Pre operative T1 weighted MRI of the

orbit with contrast: (A), (B) Axial views. (C), (D)

Coronal views. Note the mass effect is present on

the left lamina papyracea without intraconal invasion.

The tumor extends to the maxillary sinus and it is in

close relation to the skull base.

Figure 3. Post operative T1 weighted MRI: (A), (B)

Axial views. (C), (D) Coronal views. Sinuses are open

and there is no evidence of recurrent at 27-months

after the surgery.

Figure 2. Endoscopic intraoperative view with 0

degree endoscope (A, B and C) and 45 degree

endoscope (D). (A) Tumor is seen between the

inferior turbinate and septum. (B) Tumor extending

towards the ethmoid and frontal sinus recess. (C)

Cavity after the resection. (D) Frontal sinus.

CORRESPONDING AUTHOR

Pediatric sinonasal non-Ewing’s sarcoma: case report and literature review

Roshni V. Khatiwala1, Maria Peris-celda MD PhD2, Tyler J. Kenning MD2, Matthew Adamo MD2, Carlos D. Pinheiro-Neto MD PhD3

1 Albany Medical College, Albany, New York

2 Department of Neurosurgery, Albany Medical Center, Albany, New York.

3 Division of Otolaryngology / Head and Neck Surgery, Department of Surgery, , Albany Medical Center, Albany, New York.

References

1. Huh, W.W., et al., Pediatric sarcomas and related tumors of the head and neck. Cancer Treat Rev, 2011. 37(6): p. 431-9.

2. Shapiro, N.L. and N. Bhattacharyya, Staging and survival for sinus cancer in the pediatric population. Int J Pediatr

Otorhinolaryngol, 2009. 73(11): p. 1568-71.

3. Gerth, D.J., J. Tashiro, and S.R. Thaller, Pediatric sinonasal tumors in the United States: incidence and outcomes. J

Surg Res, 2014. 190(1): p. 214-20.

4. Lacovara, J., K. Patterson, and G.H. Reaman, Primary nasal chondrosarcoma. The pediatric experience. Am J Pediatr

Hematol Oncol, 1992. 14(2): p. 158-62

5. Shweikeh, F., et al., Brain metastasis in bone and soft tissue cancers: a review of incidence, interventions, and

outcomes. Sarcoma, 2014. 2014: p. 475175.

6. Holsinger, F.C., et al., Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year

multi-institutional review. Ear Nose Throat J, 2010. 89(11): p. 534-40

Carlos D. Pinheiro-Neto, MD PhD

Division of Otolaryngology – Head & Neck Surgery

Albany Medical Center

47 New Scotland Avenue MC-41

Albany, NY 12208

Phone: (518) 262-5575

Fax: (518) 262-5184

Email: pinheic@mail.amc.eduwww.amc.edu/cranialbase

Accurate diagnosis of this sinonasal sarcoma

was made possible by frequent CT scans, which

has long been utilized as an optimal radiologic tool

to assess and diagnose pediatric craniofacial

sarcomas, most notably those arising in the nasal

cavity2. Sinonasal tumor resection has been

correlated with a high incidence of post-surgery

survival3. However, the location of the tumor may

be related to different outcomes– it has been

noted that sarcomas in the posterior nasal cavity,

nasopharynx, and sphenoid sinus often have

worse outcomes than patients with sinonasal

sarcomas of other locations4. This is primarily due

to the difficulty in excising tumors from those

specific locations in the nasal cavity. Fortunately

the tumor in this case was completely resected

using endoscopic sinus surgery.

It is possible that by the time of diagnosis, the

sinonasal malignancies may have already started to

invade the skull base. About 3% of all brain

metastases can be attributed to sarcomas5. Due to

the physical proximity of sinonasal sarcomas to

the skull base, it is recommended to monitor

patients routinely for recurrence and to reduce the

risk of brain metastases.

A B

C D

A

C D

B

The patient developed forehead pain due to left

frontal sinusitis approximately one year later,

which did not improve with maximal medical

therapy. The patient underwent endoscopic sinus

surgery for frontal sinusotomy one year after the

first surgery. During that procedure, samples from

anterior skull base, sphenoid sinus and orbital

mucosa were taken. Specimens showed no

evidence of tumor, though there was some

thickening of the left medial rectus and superior

oblique as compared to the contralateral side. The

patient is currently free of disease, 27 months

after surgery (Fig. 3).