Tongue Carcinoma in a Patient with Down’s Syndrome

Tongue carcinoma
Pre-op picture. Source: World Journal of Surgical Oncology.

This article describes an unusual case of tongue carcinoma in a 27-year-old male with Down’s syndrome.

He presented to the hospital with complaints of ulceration on the left half of the tongue. In addition, he also reported excessive salivation. He denied smoking or tobacco consumption and did not give any history of a similar incident in his family.

On examination, there was a 4×3 cm ulcerated lesion on the lateral aspect of the tongue. The doctors did not notice any associated bleeding or dental involvement. They also did not find any palpable lymph nodes in the sublingual or submandibular regions.

Tongue Carcinoma on Biopsy

Baseline investigations like a complete blood count, etc., and a chest x-ray came out normal. To establish the underlying pathology, doctors proceeded with a biopsy of the lesion. Results revealed squamous cell carcinoma of the tongue, which was too poorly differentiated. Hence, doctors planned for his surgery.

Doctors did a hemiglossectomy without removing any of the neck lymph nodes. Post-operative histopathology confirmed a squamous cell carcinoma along with negative tumour margins. To minimize the risk of recurrence, doctors decided on post-op irradiation of the region which provided good loco-regional control.

Tongue Carcinoma Appears again

Although radiotherapy provided good control in the initial post-op period, the patient presented with an ulcer on the same side of the tongue three months later. This time again, a biopsy of the lesion revealed a squamous cell carcinoma. However, the patient refused to go for another surgery and, as an alternative, was put on chemotherapy by the doctors. He received two cycles of chemotherapy, after which he refused further chemotherapy as well. This left doctors with palliative care as the only option.

Palliative Care

For nutritional purposes, doctors performed a gastrostomy. They also did tracheostomies as part of palliative care. However, the patient died within three months of the recurrence.

Down’s Syndrome and Tongue Cancer: Is it Unusual?

It is quite interesting to note that people with Down’s syndrome are generally protected against cancer. The reason lies in the extra copy of the 21st chromosome. It carries a lot of extra tumour suppressor genes that inhibit cell growth and proliferation. Hence, people with this syndrome rarely present with cancer, much less carcinomas of the mouth. The incidence of tongue cancer in such patients is close to nonexistent.

Another reason for low rates of cancer in people with Down’s syndrome is the augmented tendency for the cells to undergo apoptosis. Although not very clear, most genetic experts believe it happens due to genetic effects too. Hence, people with this syndrome have an increased probability of cell death rather than cell growth. That explains how they are protected against cancer.

Our Case and Its Poor Prognosis

First, the described case is unusual. Second, the recurrence and rapid deterioration of the patient make it even rarer.

Generally, people with such chromosomal anomalies have a good prognosis for cancers. However, because people with Down’s syndrome have a large tongue in proportion to their mouth, it makes complete resection of the tumour difficult. In our case, doctors did achieve a good resection with negative tumour margins. Moreover, they also put the patient under radiotherapy for efficient loco-regional control. Despite the preventive measures, local recurrence within just three months followed by such rapid deterioration of the patient brings a lot of new questions to the table.


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