Key clinical massage
Surgeons and clinicians may face cases during their career life when
there are no straight forward guidelines available for the management
due to little cases reported throughout the literature.
BackgroundMore than 170,000 Patients newly diagnosed with laryngeal cancer cases
and up to 90,000 death annually, that account for 1-5% of all cancers
and deaths annually 1,2, squamous cell carcinoma
accounts for 85-95% of all laryngeal cancers, it is more common in
males and multiple risk factors have been linked to laryngeal cancers,
tobacco and alcohol being most related.1,3,4
With the majority of laryngeal cancers, a raise from squamous
epithelium, small percentage develops from other laryngeal
tissues.1
Radiotherapy, endolaryngeal excision and open surgery all are accepted
modalities in the treatment of early-stage glottic cancer, with
advantage for endolaryngeal excision and radiotherapy for voice function
preservation, with a trend away from open surgery, and no significant
difference in survival between radiotherapy and open
surgery.4
Radiotherapy alone or in combination with chemotherapy proven to be
effective treatment modality of laryngeal cancer in early and advance
stages, early laryngeal cancers are treated with radiotherapy
alone5,6, advance cancers require a combination of
both radiotherapy and chemotherapy7, with adverse
impact on the voice caused by glottic cancers.8
There is only one study comparing radiotherapy and open surgery.
However, the interpretation of its findings was limited due to concerns
over the study methodology and the adequacy of treatment
regimens.4
Case presentation A 59-year-old male patient, known to have hypertension and type II
diabetes mellitus on oral hypoglycemic agents, with a smoking history of
40 years, smoking index 1600, the patient presented to emergency
complaining of inspiratory stridor that started 7 days prior to ED
presentation and worsened overtime till he seeks medical advice, Patient
gave a history of hoarseness of voice for 5 months duration, weight loss
of more than 10 kilograms (22 pounds) over the past 6 months, no family
history of malignancy, unremarkable past surgical history, Fiberoptic
was done in the Emergency department (ED); it revealed an exophytic
ulcerated mass occupying the left vocal cord partially and compromising
the airway, a right vocal cord can be seen moving and fullness of left
pyriform fossa, the patient admitted to the surgical intensive care unit
(SICU) for airway observation and further evaluation.
Neck and thorax vomputed tomography (CT) scan with contrast(Figure 1) showed “heterogeneously enhancing mass lesion in the
left side of the larynx predominantly involving glottic compartment and
measuring approximately 1.8X 1.5 X 1.1cm in size. The epicenter of this
mass lesion appears to be in the left vocal cord which extends
anteromedially causing partial compromise of the airway. There is an
extension of the lesion into the left aryepiglottic fold. Superiorly
this lesion appears to extend into the supraglottic region and reaching
up to the lower part of the left pyriform sinus. Inferiorly this mass
lesion extends into the upper part of the infraglottic compartment.
Laterally extralaryngeal extension into the paralaryngeal space seen
abutting the thyrohyoid membrane”
The patient underwent microlaryngoscopy (Figure 2) with biopsy and
debulking of the mass, the operative findings were fungating mass
arising from supraglutic region, involves in left vocal cord, sparing
the anterior commissure, left arytenoid, and left pyriforn fossa, and
the far posterior aspect of left vocal cord looked spared, sample sent
for histopathology, pathology result was “fragments of partly ulcerated
squamous mucosa with extensive underlying infiltration by a malignant
neoplasm, composed of pleomorphic spindle cells, interspersed by
numerous histiocytes”. The malignant spindle cells exhibit frequent
mitosis, including atypical mitosis, immunohistochemical stains were
positive for SMA, Vimentin and CD68 (in histiocytes), findings
consistent with Undifferentiated pleomorphic sarcoma (Figures
3,4) .
Other workups for metastasis carried out and the patient was free of
distant metastasis, the patient discussed by the multidisciplinary team
(MDT), plan made for total laryngectomy followed by radiotherapy, he
underwent total laryngectomy with neck dissection, frozen sections done,
all margins were negative for malignancy.
Histologically, the debulked fungating tumor showed partly ulcerated
squamous mucosa with extensive underlying infiltration by a pleomorphic
malignant spindle cell neoplasm, exhibiting frequent mitoses. There was
no surface squamous dysplasia and no histological evidence of the tumor
originating from the surface. The tumor was subjected to a wide panel of
immunostains, but no definite cell lineage was appreciated.
Areas of smooth muscle actin (SMA) and vimentin positivity were present,
but various other markers, including cytokeratins, P40, P63, desmin,
CD34, CD31, MyoD1, WT1, calretinin, Sox10, S100, MDM2, and CDK4; were
all negative. Scattered CD68 positivity highlighted histiocytes
interspersed amongst the tumor cells. The morphological features,
coupled with the lack of specific immunohistochemical markers, were
consistent with a diagnosis of undifferentiated pleomorphic sarcoma.
Examination of the subsequent laryngectomy specimen revealed residual
Grade 2 (following the French Federation of Cancer Centers Sarcoma Group
(FNLCC) grading system) tumor in the left vocal cord, measuring 1.5cm in
maximum dimension, with negative margins.