A 51-year-old woman presented to the emergency department with a 2-month history of headache.
The CT scan showed a 38 x 21 mm lytic lesion involving the left temporal quadrant immediately above the mastoid.
As personal history, thermocoagulation of a skin lesion on the same region in another hospital center stands out, currently without apparent skin involvement.
After performing a complete body bone extension study and chest, abdomen and pelvis computed tomography (CT), which were negative, surgical excision of the lesion was performed in collaboration with the Neurosurgery Service.
The lesion was removed with circumferential en-bloc craniectomy including temporal muscle and fascia, with a macroscopic margin of 1 cm. The apparently conserved, durable aspect of the lesion had no underlying appearance.
A 5-cm acrylic repair was made for the bone defect and direct closure of the scalp was performed.
1.
The mass was soft and whitish and eroded external and internal table of the temporal bone.
1.
The pathological study showed a well-defined multiple solid nest neoplasia separated by fibrous tissue.
Inside these nests an atypical epithelium was found with frequent mitoses arranged around central areas formed by keratin material.
All these characteristics represent a malignant tumor originated in the hair follicle and in particular a trichofollicular carcinoma.
1.
As a consequence of the most probable origin of the tumor, the history of skin lesion and the fact that the scalp was not removed in the first intervention, we performed a wide revision of the bed and resection of the island of scalp.
The intervention was programmed by the Plastic Surgery Department two months after the first operation, but the day before the scheduled date the patient was admitted for hemoptysis in the Pneumology Department with a negative evolutional CT.
Given the time elapsed, a new control cranial CT was performed, which reported the presence of post-surgical changes without signs suggestive of local recurrence or lymph node dissemination.
Finally, three months after the first intervention, the patient underwent a new surgery, with wide excision of the scalp and exposure of the previous surgical bed.
Intraoperatively we observed the presence of a new, small, 1 cm lesion, located 1 cm from the posterior edge of the craniectomy and without contiguity with the previous one, with preserved external table but apparent erosion internal table.
We removed the acrylic plasty, extended the craniectomy, performed a circumferential dural resection with margins and repair of the meningeal with a Neuropicture® plasty.
The bone and final defect, 12 x 7 cm in diameter, was cutaneously removed by Plastic Surgery with a rectus abdominis muscle free flap based on the inferior epigastric artery (TRAM) and skin free muscle graft.
The location of the tumor in the union of the middle and posterior third of the skull base made it more complex to have recipient vessels for the flap.
Finally, end-to-end anastomoses were performed to the superior thyroid artery, which was subsequently fixed to arrive at the flap and the external jugular vein.
Pathological examination of the excised piece revealed an unusual ganglion due to a tumor of the same characteristics as authentic, i.e. tricofollicular carcinoma in internal table, diploe and dura mater,
A third surgical intervention was necessary because a new anterosuperior parietal lesion was detected without continuity with the previous craniectomy in the control scanner in less than 1 month of evolution.
We could access the upper edge of the previous craniectomy through the incision of the superior edge of the TRAM flap and its extension to the scalp over the superior temporal line.
Located open craniectomy 4x5 cm anterosuperior to include the new lesion and the underlying dura with margins.
Closure was performed with another Neurop® plasty and acrylic cranioplasty only in the new bone defect (the previous defect was completely covered by the TRAM flap).
1.
Both diploe and dura mater were diagnosed for tricofollicular carcinoma, with negative resection margins.
The cranial and thoracic control scanner 3 months after the last intervention showed no recurrence of local disease and detected the presence of two apical pulmonary nodules that were removed by videothoracoscopy.
The pathology report showed lung metastases with histological image similar to previous biopsies.
Lung metastases are defined as primary trichofollicular carcinoma of the scalp.
As adjuvant treatment the patient received local radiotherapy centered on the surgical bed and the flap with very good adaptation and evolution: external radiotherapy with intensity modulation (IMRT) for 1 month and a half at a rate of 2 sessions per week.
Adjuvant chemotherapy was also administered: 6 cycles with CDDP-5FU (cisplatin with fluorouracil) and another 4 cycles of choline-taxol with good response.
Two and a half years after the diagnosis of the primary tumor and dissemination of this last cycle of chemotherapy, there are no signs of tumor, lung or abdomen or pelvis recurrence in CT.
