DOI No.: 10.21176/ ojolhns.0974-5262.2015.09.

INSULAR CARCINOMA OF THYROID UNUSUAL VARIANT

OF THYROID MALIGNANCY
*Dipak Ranjan Nayak, *Asheesh Dora, **Ranjani Kudva, ***Shekar Patil
ABSTRACT

INTRODUCTION:

A rare case of histologically distinct aggressive

Thyroid malignancy is one of the common endocrine

thyroid cancer that has been termed recently as poorly

malignancies seen in clinical practice and its incidence

differentiated (Insular) thyroid cancer is reported. The

has progressively increased in the last three decades all

FNAC was suggestive of a follicular lesion. CT scan was

over the world . The thyroid cancer originating from the

strongly suggestive of malignancy. Total thyroidectomy

with bilateral MRND and central compartment clearance
was done and was confirmed histopathologically as

follicular cell has been traditionally classified further into

well

differentiated

(papillary,

follicular

etc.)

and

Undifferentiated/Anaplastic (Patel & Saha 2006) . About

insular carcinoma of thyroid. The patient was advised

EBRT with radioiodine ablation. The patient reported back

Vol.-9, Issue-I, Jan-June - 2015

7 months after with dysphagia and was found to have

90% of thyroid malignancies are well differentiated thyroid

3

carcinomas (WDTCs) with current modality of treatment .

recurrence in the cervical esophagus and superior

Undifferentiated (Anaplastic) thyroid carcinoma (UDTC) is

mediastinum along with lung and liver metastases on

a rare presentation with an aggressive clinical course and

hypopo-haryngoscopy and CT scan and underwent 5

with early distant metastases. There is one more group of

cycles of palliative chemotherapy. On last follow-up there

thyroid carcinoma which neither can be classified as

was gross regression of tumor and the swallowing was

WDTC nor UDTC. They are classified as poorly

improved. Relevant literature has been reviewed and the

differentiated thyroid carcinoma (PDTC)

role of chemotherapy to improve survival has been

Saha described poorly differentiated thyroid carcinoma as

discussed.

a group of thyroid cancer that include carcinomas of

Key words: Insular thyroid cancer, poorly

differentiated thyroid cancer, chemotherapy.

follicular thyroid epithelium that keep ample differentiation

. Patel and

to generate spread of tiny follicular structures but are free

from usual morphological picture of papillary and follicular

Address for correspondence:

carcinoma although they have some thyroglobulin. They

Prof.Dipak Ranjan Nayak

also broadly divided these tumors into Insular and Other

Department of ENT-Head & Neck

(Large cell) type . This article discussed about the role of

Surgery, Kasturba Medical College,

multimodal treatment in these cases especially adjuvant

Manipal. E-mail: drnent@gmail.com

28

chemotherapy to improve survival and quality of life.

*Department of ENT-Head & Neck Surgery, **Dept. of Pathology, Kasturba Medical college, Manipal.Karnataka, India, ***Consultant

Medical Oncologist, HCG, Bengaluru, Karnataka, India

DOI No.: 10.21176/ ojolhns.0974-5262.2015.09.1

CASE REPORT:

The final histopathology report showed sheets and

A 28 year old female, with no co-morbidities,

islands of malignant cells with focal peritheliomatous

presented with swelling in the anterior neck of 2 months

pattern with high N:C ratio, hyper chromatic round to

duration. Swelling was insidious in onset and gradually

ovoid pleomorphic nuclei with scanty cytoplasm and

progressive in size. History of any breathing difficulty and

coarse chromatin seen infiltrating into adjacent thyroid

dysphagia were not reported. Ultrasonography of neck

tissue. It was reported as poorly differentiated Carcinoma

showed an enlarged hypo-echoic nodule in the right lobe

(Insular carcinoma of thyroid). Immunohistochemistry and

of thyroid (5cm x 3cm x4cm) with enlarged level VI lymph

immunofluorescence was done and tumour cells were

node on the right side. Fine needle aspiration cytology

diffusely positive for cytokeratin and focally positive for

was done and was suggestive of follicular lesion of thyroid

synaptophysin. Cells were negative for Chromogranin and

and suggested a frozen section.

LCA. Ki67 was

Contrast enhancement CT of the neck and thorax

positive for 30-40% cells.

was done as the clinical picture was suggestive of

Radio nuclide scan was done after 1 month which

malignancy and suspicious nodes on ultrasound. On

showed traces of thyroid tissue in the thyroid bed and

the scan there was heterogeneously enhancing lesion

Patient was advised and counseled for radio iodine

in the right lobe of thyroid and also involving the

ablation and also EBRT in view of gross infiltration into

isthmus with infiltration into the right sided strap

the surrounding structures which she wanted to receive

muscles with enlarged discrete lymph nodes at level II

at his native place. Patient was lost on follow up and

and VI on right side. Lesion was abutting the internal

presented 7 months later with dysphagia of insidious,

jugular vein and carotid artery. There was no evidence

of the involvement of lung or mediastinum and the
patient was clinically staged as T4 N2Mx.

gradually progressive and more for solids than liquids.

She could not undergo for neither radio iodine ablation
nor EBRT for financial and personal reason. A barium

Patient was counselled about the condition. All

swallow was done which showed eccentric filling defect

haematological parameters were normal including the

with mucosal irregularity at the level of C7-T2

infiltration of the tumor into the strap muscles and trachea

pushed toward the opposite. Frozen section was sent
from the right lobe of thyroid and also the strap muscles
and was suggestive of malignancy with muscle infiltration

Patient later underwent hypopharyngoscopy on

which two ulcerative lesions were found at the level of
15cms and 17cms from the incisors. Separate biopsies
were taken from the lesions and were sent for HPE.
Biopsy was reported as consistent with malignancy and

suggestive of follicular neoplasm. Patient underwent total

infiltration from the insular carcinoma thyroid. Patient

thyroidectomy

neck

was not willing for any surgical procedures and advised

dissection and central compartment clearance with

a metastatic workup followed by palliative chemo at a

preservation of sternocleidomastoid muscle and IJV. Strap

nearby place. A Radio Iodine scan was repeated that

muscles were resected. Left inferior parathyroid gland was

did not show any uptake. CECT showed cervical &

preserved.

mediastinal Lymph node with lung and liver metastasis.

with

bilateral

modified

radical

29

- 2015

thyroidectomy. Intra operatively there was a gross

Issue- JanJune
I,

planned for a frozen section and then proceeds for total

suggestive of esophageal involvement.

Vol.9,

thyroid profile and routine investigations. Patient was

DOI No.: 10.21176/ ojolhns.0974-5262.2015.09.1

She was started on oral Sorafenib tosylate. 5months later

Histopathological diagnostic criteria also known as the

a PET scan was done and it showed progressive disease

Turin criteria were published by Volante et.al (2007)

and she was started on Gemox Regimen (Gemcitabin

which include: (a) the presence of solid/trabecular/

1gm / M D1, D8 + Inj. Oxaliplatin 135 mg / M ). After 3

insular pattern of growth. (b) The absence of the

cycles, on assessment with CT scan Thorax showed

conventional nuclear features of papillary carcinoma

significant regression in mediastinal Lymph node and lung

(c) the presence of at least one of the following

microscopic features: convoluted nuclei; mitotic

and

liver

lesion.

Patient

completed

cycles

of

chemotherapy and there was poor tolerance. So was

again started on oral Sorafenib tosylate. Patient has
significant symptomatic improvement including better
swallowing at 1 year 4 months.

activity e 3 x 10HPF; or tumour necrosis .

The FNAB can give useful information but not a
definitive preoperative diagnosis. The thyroglobulin (TG)
and thyroid transcription factor1 (TTF1) are positive on

DISCUSSION:

immunohistochemical analysis as these tumors are of

Insular carcinoma is an aggressive form of thyroid

cancer that has been included recently under poorly
4

differentiated thyroid cancer . Sakamoto et al (1983)

coined the term of poorly differentiated thyroid
carcinoma for the histology variants that are showing

follicular origin and p53 may stain positive from 0-38%

2

cases of insular carcinoma . Due to high incidence of

regional metastases, a total thyroidectomy with central
compartment and bilateral modified neck dissection
2

should be considered . Surgery followed by radioiodine

non-papillary/non-follicular growth pattern with poor

therapy is considered to be the standard treatment

prognosis. Five-year survival for PDTC is less than

protocol even for the PDTCs. There is data that shows

65% in contrast to a WDTC, which is more than 95% .

Carcangiu et al. also proposed a similar description for

that Insular variant has the ability to take up radioiodine in

up to 85% of cases and sometimes even better compared

Vol.-9, Issue-I, Jan-June - 2015

aggressive thyroid malignancies and used the term

to the predominant solid or trabecular growth patterns .

initially

They

The efficacy of External beam radiotherapy has also been

reintroduced the term insular carcinoma to the

described. The external beam treatment for PDTC should

histological type characterized by the presence of

be considered in T3N0M0tumours, all T4 and in Any

insulae which included small cells with round to oval

TN1M0tumours. Chemotherapy in patients with PDTCs

hyper chromatic nuclei, increased mitotic activity over a

ought to be considered individually. Use of methotrexate,

necrotic background. The term insular was used to

vinblastine, doxorubicin, and bleomycin in monotherapy

describe these tumors because the cellular appearance

has been proposed. Combination therapy composed of

was similar to that seen in the insular type of carcinoid

chemotherapy and external radiotherapy is considered

describes

by

Langhans

in

1907.

tumors. . In 2004 WHO defined PDTC as a neoplasm

experimental . In the present case Sorafenib to sylate

developing

was tried with limited response, so Gemcitabin and

from

thyroid

follicle

cell,

presenting
both

Oxaliplatin was tried with good response but had poor

morphological and biological intermediate behavior

tolerance after 5 cycles and has now being maintained on

between well-differentiated thyroid carcinomas and

Sorafenib. There was good symptomatic relief and

restricted

differentiation

to

it

and

having

undifferentiated carcinoma . PDTC may also develop in

the persistent well differentiated thyroid carcinoma.

30

excellent over all response.

DOI No.: 10.21176/ ojolhns.0974-5262.2015.09.1

CONCLUSIONS
Poorly differentiated thyroid carcinoma is a condition
yet to be explored. The low incidence, aggressive clinical
course and fatal outcome due to very low five year
survival rate pose a greater difficulty in

Illustrations:

Fig. 3(a) H&E x 40- shows a tumor composed of small cells

arranged in solid, trabecular and insular pattern (b) H&E x
20- Focal peritheliomatous pattern of tumor cells seen.

thorough understanding and treating this condition

satisfactorily.
DISCLOSURES
Fig.1: CECT neck (a)axial cut, (b)showing the tumor which is
infiltrating the Left Sided strap muscles .The trachea has
been pushed laterally.

(a) Competing interests/Interests of Conflict- None

(b) Sponsorships - None

(c) Funding - None
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