Abstract cervical to 2nd dorsal vertebrae measuring 8*4.5*6

Abstract

Minor
Salivary Gland tumours are rare,
representing 2% of the head and neck tumours
and less than 1% of hypopharyngeal cancers. The most common subtype of minor
salivary gland tumours is adenoid cystic
carcinoma. Although there are several case reports discussed it, there are only
two reports discussing hypopharyngeal
presentation in the literature, and they were treated by surgical resection.
Despite that, our case report still to be unique as it presents a case of
irresectable locally advanced tumour
subjected to a trial of induction
therapy.

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Introduction

Anatomically,
the palate is the commonest site of minor salivary gland tumours, followed by paranasal sinuses, nasal
cavity and the larynx.1 Adenoid cystic carcinoma (AdCC) is
the most common subtype of minor salivary gland tumours
representing around 10% of all malignant salivary gland tumour.2 The main cornerstone of treatment is the surgical resection while the role
of radiotherapy and chemotherapy are still controversial.3 Several prognostic factors have
been identified such as free resection margin, nodal negativity and negative
neural invasion.3

However,
AdCC of the hypopharynx is extreme rare
disease, thus, extrapolating the clinical behaviour
of laryngeal and pharyngeal counterparts is acceptable. 2 Generally,
the disease is slowly growing but it has extensive invasiveness potential and
high recurrence rate following resection. Likely as its counterparts in hard
palate, adenoid cystic carcinoma is characterized by poor responsiveness to
chemotherapy and radiotherapy. 2, 3

The
driver mechanism of adenoid cystic carcinoma is unknown. They arise between the
fifth and sixth decade of life as submucosal mass and hence delayed diagnosis
until advanced stages. 3

Case report

                A
59-years old male smoker was presented with a history
of cervical lymph node enlargement, stridor managed by urgent tracheostomy.

                CT
and MRI scanning revealed, large retro-laryngeal mass extending from the level
of 4th cervical to 2nd dorsal vertebrae measuring 8*4.5*6
cm in maximal Cranio-caudal, transverse and anteroposterior dimensions,
respectively. The mass is infiltrating the internal Jugular, the thyroid and
prevertebral fascia. CT chest and the abdominal
US excluded metastatic spread.

His endoscopy
revealed a large post-cricoid mass disturbing the laryngopharyngeal anatomy. Multiple biopsies were taken and
examined pathologically
showing low-grade
uniform cells with adenoid cystic differentiation. Immune staining revealed
positive CK, CD117 and S100 beside negative TTF-1 supporting the diagnosis of
AdCC.

                Due
to the rarity of the disease and lack of
supporting guidelines, the patient received a trial of Docetaxel 80mg/m2
D1, Cisplatin 100mg/m2 D1 and 5-fluorouracil 1000 mg/m2 D1-4
for two cycles. The first cycle was well-tolerated, however, in the eighth day following the second cycle, he
developed uncontrollable neutropenia, mucositis and diarrhoea that is
complicated by death.

Discussion

            The most
common tumours in the hypopharynx are the
squamous cell carcinoma (90%) followed by other histological types. AdCC
usually grows as a submucosal mass that
is barely detected at earlier stages.1, 2 Adenoid cystic carcinoma occurs in old age
groups with an equal male to female
distribution. Symptoms are usually related to dysphagia,
especially for solids.

Figure 1; Sagittal T2W MRI –  infiltration of the prevertebral fascia

AdCC usually recurs post-resection especially in patients with narrow
resection margins. Several factors have been linked to improved 5-years
survival rate; negative resection margin, nodal negativity and absence of
neural invasion. Although surgical resection of AdCC is considered the standard
whenever possible, other modality of treatment such as radiotherapy and
chemotherapy still controversial. Although several publications pointed to the
use of palliative chemotherapies; either taxane-based or anthracycline-based combination, none of them showed any survival
benefit. 4, 5

Few authors
reported cases of AdCC in rare anatomical sites such as larynx, however, only
two authors reported two cases of hypopharyngeal AdCC which were detected in an
earlier stage and treated by surgical
resection. 3, 6 Our case is still to be unique since it
represents a locally irresectable disease in the hypopharyngeal region and is
treated by docetaxel, cisplatin and 5-fluorouracil combination. Despite the
negative outcome; death of the patient, it is irrational to judge a regimen’s
efficacy and response from one case study.

Conclusion

                Minor
salivary gland cancer is one of the rare head
and neck neoplasms. Adenoid cystic carcinoma is the most common
histological subtype to be seen within that group. The commonest site for AdCC is the hard palate,
paranasal sinuses and nasal cavity. Other sites such as larynx and hypopharynx
are rare to be seen. Our case is unique
and distinct since it represents a patient with irresectable late stage AdCC of the hypopharynx.
Unfortunately, our trial of docetaxel cisplatin and 5-fluorouracil failed to
show any response in that patients. Further studies of such disease are necessary for the proper definition of treatment guidelines.

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