A 51-year-old woman came to the emergency department with headache of 2 months' duration. The scan showed a lytic lesion measuring 38 x 21 mm involving the left temporal calvarium immediately above the mastoid. The patient's personal history included thermocoagulation of a skin lesion in the same region in another hospital, currently without apparent involvement of the skin. After performing a full body bone extension study and computed tomography (CT) of the thorax, abdomen and pelvis, which were negative, we proceeded to surgical excision of the lesion in collaboration with the Neurosurgery Department. The lesion was removed by circumferential en bloc craniectomy including the temporalis muscle and its fascia, with a macroscopic margin of 1 cm. The underlying dura mater had an apparently preserved appearance, without infiltration. A 5 cm acrylic plasty was cast for the bony defect and direct scalp closure was performed.
The excised mass was soft and whitish in consistency and eroded the external and internal table of the temporal bone.
Pathological examination showed a neoplasm in multiple well-defined solid nests separated by fibrous tissue. Inside these nests, atypical epithelium with frequent mitoses was found arranged around central areas formed by keratic material. All these characteristics represent a malignant tumour originating in the hair follicle and specifically a trichofollicular carcinoma.
As a consequence of the most probable origin of the tumour, the history of skin lesion and the fact that the scalp had not been removed in the first operation, we decided to extend the surgery and perform a revision of the bed and resection of the scalp island. The operation was scheduled by the Plastic Surgery Department 2 months after the first operation, but the day before the scheduled date the patient was admitted to the Pneumology Department for haemoptysis, with negative fibrobronchoscopy and CT scan showing pleural thickening, and therefore a follow-up examination was recommended. Given the time that had elapsed, a new control cranial CT scan was performed, which revealed the presence of post-surgical changes with no signs suggestive of local recurrence or lymph node dissemination.
Finally, 3 months after the first operation, the patient underwent another operation with extensive resection of the scalp and exposure of the previous surgical site. Intraoperatively we observed the presence of a new lesion of small size, 1 cm, located 1 cm from the posterior edge of the craniectomy and without contiguity with the previous one, with preserved external table but eroding internal table and with apparent dural infiltration. We removed the acrylic plasty, extended the craniectomy, performed circumferential dural resection with margins and repaired the meninx with Neuropatch® plasty. The final bone and skin defect, 12 x 7 cm in diameter, was reconstructed by Plastic Surgery with a free flap of rectus abdominis muscle based on the inferior epigastric artery (TRAM) and free skin grafting of partial skin over the muscle. The location of the tumour at the junction of the middle and posterior third of the skull base made the availability of recipient vessels for the flap more complex. Finally, the anastomoses were made end-to-end to the superior thyroid artery, which was subsequently rotated to reach the pedicle of the flap and the external jugular vein.
The anatomopathological study of the excised piece showed infiltration by a tumour with the same characteristics as the primitive one, i.e. a trichofollicular carcinoma in the internal table, diploe and dura mater, with free borders, which curiously did not infiltrate the scalp, epidural tissue or local ganglia.
A third surgical intervention was necessary because a new antero-superior parietal lesion without continuity with the previous craniectomy was detected in the control scan in less than 1 month of evolution. We were able to access the upper edge of the previous craniectomy through the incision of the upper edge of the TRAM flap and its extension towards the scalp on the upper temporal line. We extended the craniectomy 4x5 cm antero-superiorly to include the new lesion and the underlying dura with free margins. Closure was performed with another Neuropatch® plasty and acrylic cranioplasty only on the new bone defect (the previous bone defect was completely covered by the TRAM flap).
Both diploe and dura were infiltrated by trichofollicular carcinoma, with negative resection margins.
The control cranial and thoracic scan 3 months after the last operation showed no recurrence of local disease and detected the presence of 2 apical pulmonary nodules which were removed by videothoracoscopy. The anatomopathological report was of pulmonary metastases with histological image similar to that of the previous biopsies. In view of this, we assumed the diagnosis of the lung as a metastasis of primary trichofollicular carcinoma of the scalp.
As adjuvant treatment, the patient received local radiotherapy centred on the surgical bed and the flap with very good adaptation and evolution: intensity modulated external radiotherapy (IMRT) for 1 month and a half at a rate of 2 sessions per week, using high-energy photons generated by the linear accelerator (SYNERGY®) with a total dose of 12,600 cGy.
He also received adjuvant chemotherapy: 6 cycles of CDDP-5FU (cisplatin with fluorouracil) and another 4 cycles of carboplatin-taxol with a good response.
Two and a half years after the diagnosis of the primary tumour, and having finished this last cycle of chemotherapy, there are no signs of tumour recurrence, pulmonary, or dissemination to the abdomen or pelvis in the CT scan.
