Template provided by: “posters4research.com”

METASTATIC CONVENTIONAL PRIMARY

CHONDROSARCOMA OF THE HYOID

Mary Ondinee U. Manalo, MD, Fellow-in-Training, Section of Medical Oncology, UP-Philippine General Hospital

Gracieux Y. Fernando, MD, Consultant, Section of Medical Oncology, UP-Philippine General Hospital

FIGURES

THE CASE

DISCUSSION

THE CASE INTRODUCTION

In conclusion, according to

the published clinical series,

head and neck CHS is usually a

low grade neoplasia with a high

propensity for local recurrences

that is commonly treated by

surgery alone. The case

reported in the present article

reveal a high grade tumor that

metastasized early in its clinical

course. Surgery was not an

option so palliative

chemotherapy was attempted

but was not successful.

Outcomes of CHS are

unpredictable, so strict follow-

up is required. //

CONCLUSION

Chondrosarcoma (CHS) is classified by the World

Health Organization as a malignant tumour

characterized by the formation of cartilage, but not of

bone, by tumour cells. Chondrosarcoma is the third

most common primary malignancy of bone after

myeloma and osteosarcoma. They are rare in the head

and neck region which accounts for less than 1 per

cent. Most chondrosarcomas of the head and neck

region occur in the maxilla; others are found in

descending order of frequency in the ramus, the body

of the mandible, the nasal septum, and the paranasal

sinuses.

The etiology of these tumours is unknown.

However, they are formed from cartilage in tissues not

normally harbouring cartilage or, secondly, from the

cartilage cap of exostosis or enchondromas. Vestigial

rests of multipotential differentiation of mesenchymal

cells may be the forerunner.

Chondrosarcomas of the head and neck have been

reported in patients ranging in age from 17 months to

75 years. The peak age of incidence is the third to the

sixth decade. There does not seem to be a sex

predilection. The tumours show a less aggressive

course when found in the long bones rather than when

found in the head and neck as the latter have a greater

rate of growth, recurrence, and metastasis. They

usually present as painless swellings.

Conventional radiographic findings are usually

not pathognomonic. Single or multiple lucencies with

poorly defined borders or bone destruction with

associated calcifications or ground-glass or sun-burst

appearance, and uniform widening of the periodontal

ligament (PDL) space, may occasionally be present.

However, contemporary imaging techniques like

computed tomography (CT) scan, magnetic resonance

imaging (MRI), and fluorine-18 fluorodeoxyglucose

positron emission tomography (FDG PET) are useful to

diagnose chondrosarcoma and to differentiate this

malignancy from its benign counterpart.

A 22-year old male from the Philippines had a

seven-month history of a fast-growing anterior neck

mass (Fig 1). Thinking that it was just goiter, he did not

seek consult until a few weeks prior to admission when

he developed difficulty in breathing and swallowing.

This progressed in severity and he was subsequently

rushed to the emergency room of the Philippine

General Hospital where he underwent emergency

tracheostomy. On imaging, a heterogenous mass with

complex echoes measuring 24 x 13 x 12 cm on its

greatest diameter was seen on his neck with primary

destruction of the hyoid bone, encasing both the

internal carotids and the subclavian veins. Pulmonary

masses and nodules were seen bilaterally (Fig 2), the

largest of which measured 3.4 x 2.3 x 2.1 cm. Biopsy of

both the neck mass and the pulmonary mass revealed

grade III conventional chondrosarcoma (Fig 3).

Surgical resection was not attempted because the

mass encased major vessels and the defect will lead to

significant morbidity. Palliative chemotherapy using

single agent doxorubicin was given. The patient

received two cycles with no decrease in the size of his

neck mass. Prior to his third cycle, the neck mass

ulcerated and became infected. Due to meager

finances, he was not brought immediately for medical

consult until he developed high grade fevers and chills.

He was managed in a local hospital but he later

succumbed to septic shock. Wound and blood culture

isolated Burkholderia cepacia.

CHS is a slow growing malignant tumour with

hyaline cartilaginous differentiation, accounting for

approximately 20% of all malignant bone tumors. CHS

arising in the head and neck region is rare, accounting

only 0.1% of head and neck malignant tumours,

consequently, very few clinical series concerning the

clinicopathological features of head and neck CHS have

been published in the literature and most of them

comprise a small number of patients. In this paper, the

authors updated the review of Pontes. We reviewed all

the clinical series dealing with head and neck CHS,

summarizing the most important aspects found by each

author using PubMed and Medline.

In the review, these tumors present with a wide

range of clinical alterations, but a painless swelling is

the most common complaint. This is similar to the case

presently being reported. Microscopically, conventional

CHS constitutes the most common subtype, accounting

for approximately 90% of the cases, again was

consistent with the case being presented. The other

variants account for the remaining 10% and include

secondary, dedifferentiated, clear cell, and

mesenchymal chondrosarcomas.

The most common cause of death is related to

direct extension of the tumor to involve nearby

structures, since node and distant metastases are rarely

observed in head and neck CHS. Our patient died from

septic shock from the superimposed bacterial infection

on his necrotic tumor. We hypothesized that his tumor

probably developed necrosis that led to tumor rupture.

According to the observations in the clinical series,

when distant metastases develop, they are most likely

to be seen first in the lung. Computed incidence of

metastases is at 5.9%. In the case of the patient, when

he came to our Institution, he already had bilateral

pulmonary metastases.

Taking into account the overall biological behaviour

of head and neck CHS, the most effective treatment

modality for this neoplasia is aggressive surgery with

wide en-bloc resection. Irradiation and chemotherapy,

although variably used in cases of CHS, do not appear

to have a significant effect on survival and it is generally

accepted that these treatment modalities should be

used for palliative purpose.

According to the clinical series reviewed, the 5-year

survival rate for head and neck CSH varies between 32%

and 87.5%.

Fig 1. The patient with his rapidly enlarging

neck mass. He developed respiratory distress

and subsequently had a tracheostomy.

Fig 2. On metastatic workup, bilateral

pulmonary metastases were noted. This was

taken a week before tracheostomy.

Fig 3. Conventional chondrosarcoma on light

field microscopy.