A 17-year-old male patient was referred to our hospital in December 2013, presenting with a 4 months history of gradual flaccid paralysis at both lower limbs, associated with anaesthesia of the same area and urinary/fecal incontinence; he also complained of cough since 1 month before admission. He didn’t report any trauma and he was healthy before the onset of paralysis.
An MRI of the spine was performed in a Teaching Hospital, and it showed signal alteration on the body of T9 with pathological tissue invading the body, left peduncle, lamina and neuroforamen, compressing the spinal cord epidurally; no significant contrast enhancement. Following this investigation, in August 2013, he underwent to a neurological surgical operation on the dorsal spine (probably biopsy, but documentation not available).
When he was admitted in our hospital, he was in fair general conditions, febrile (T 38.8°C) but stable.
There was chronic anaemia but the patient has never been transfused. Another important finding has been diaphoresis: extensive sweating above all at night.
On examination there was reduced air entry at the left lung. The neurological examination showed a complete flaccid paralysis at both the legs, with sensitive and motor levels below T9-T10 and with sphincter incontinence; the rest of the neurological examination was negative. There was a deep sacral bed sore.
We performed some more investigations:
Pelvis X-Ray: normal pelvis, no signs of multiple myeloma.
Thoracic spine X-Ray: normal thoracic spine examination
Chest X-Ray: features of left extensive pleural effusion (not confirmed at U/S)
Chest U/S: extensive left lung atelectasia
FHG (full hemogram) showed: mild anemia (Hb 6.6 g/dl), WBC 9.800/dl (granulocytes 77%, lymphocytes 14%).
HIV test was negative.
Sputum for AAFB (Acid Alcohol Fast Bacilli) was not performed because the cough was non-productive).
ESR was 104 mm/1h
The other relevant laboratory tests (liver and kidney function tests, electrolytes) were within range of normality.
On 1st January 2014 we decided to start anti-TB treatment, together with large spectrum antibiotics, considering the clinical history, the involvement of the left lung and the MRI image of T9 infiltration (TB spine?) .
We have sent to Italy the neuroradiological documentation for a neuroradiology advice: it was suggested a possible neoplastic origin of the mass at T9.
We noticed a mass at the level of T9, just at the same level of the previous operation performed in another facility. The mass was looking fleshy and not containing bone tissue. Considering the rapid increase in size of the dorsal mass during the hospitalization, an open biopsy was performed (previously FNA was also performed but it was not diagnostic – skeletal muscle, and foci of mild non specific inflammation-).
The biopsy was sent to an important teaching hospital in Nairobi, and the report reads: “tissue exhibiting proliferation of spindle cells on an inflamed and necrotic background. Spindle cells show mild atypia, mitoses are not easily identified. PAS and ZN stain are negative for organisms. Conclusion: spindle cell neoplasm”.
Actually the general conditions are stable. After the biopsy, there was a progressive discharge of necrotic tissue from the wound, resulting in reduction of the dorsal mass.
Physiotherapy, which he began immediately after admission to our hospital, is still going on, and the patient can stay for some hours a day on a wheelchair, but no improvement of the lower limb paralysis was observed.
The sacral bed sore was treated with a myocutaneous flap from the gluteus, with partial improvement.
Because of the histopathology report we have decided to try to stage the neoplastic disease:
A CT scan of the chest revealed few sub-centimeter nodules in the Rt lung field. There was a 15 x 10 cm mass in the posterior mediastinum with lobulated margins. The radiologist has described the mass as invasive and extensive.
The same mass was lysing the T9 vertebra and causing anterior angulation of the spinal column. There was extension of the mass in the spinal canal from T7 to T9 causing severe cord compression and compromise.
Adjacent ribs were also lysed. On top of that the mass was also displacing the aorta anteriorly and it was invading the left pericardium. The mass also invaded the posterior lung segments (left more than right) and the left superior lung segment. It was also crossing the left diaphragm inferiorly.
The abdominal CT scan on the other hand was negative for metastases.
The conclusion of the radiologist has been: “Invasive, extensive posterior mediastinum tumour mass (known spindle cell tumour). Rt lung nodules represent metastases”.
The staging has shown us a situation which is completely beyond any possibility of treatment oncological treatment, at least in Kenya. We have consulted an oncologist in Nairobi and he has advised us on palliative care only.
We have immediately stopped the anti-TB treatment because it is useless and only dangerous for the liver of the patient.
We are now managing our client with intra venous steroids, aiming at improving appetite (now there is important anorexia) and trying to fight dyspnoea. We continue with nursing care in order to prevent worsening of bed sores and we plan to disclose the truth slowly to the patient and the relatives.
The presentation of this malignancy has been very tricky and difficult to diagnose. Unfortunately we have reached a definitive diagnosis too late for any treatment.
Still there are unsolved doubts in my mind:
spindle cell neoplasms are of epithelial origin.
Is it possible to have a primary spindle cell carcinoma from the posterior mediastinum?
Or is the primary tumour from the skin where the first operation was done?
Is it possible for a skin epithelial cancer to spread and to form such a big mass in the posterior mediastinum after involving vertebrae and spinal cord?
I know all of this is now purely academic and the reality is that a 17-year-old boy will soon die, but if any of the readers has any idea on the primitive malignancy, please let him share his views with me.
Dr Bro Beppe Gaido
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