A 16-year-old black girl with oculocutaneous albinism was referred from Maputo Hospital (Mozambique) with a diagnosis of squamous cell carcinoma of the scalp.
At the time of his home admission he presented a large lesion, ulcerative, with destruction of the left auricular pavilion and osteolysis, of progressive growth according to his 9 years of age, which had been irregularly controlled.
It requires analgesia maintained by constant pain that is exacerbated by cures.
Blood test set with hemoglobin ̈ g/dl normal; normal biochemistry; HIV negative serology; chest X-ray and abdominal ultrasound normal.
On admission he was treated with oral amoxicillin for local infection and he was cured with vaseline and metronidazole.
Family history is unknown.
In the general physical examination we found: weight 40 kg and height 163 cm; important state of malnutrition and cachexia; low muscle mass and fat; skin and mucous membrane dryness.
The auricular lesion is large, about 20 cm in diameter, with destruction as we have commented on the left auricular pavilion, which erodes the temporal bone, and with signs of suppuration.
The complementary examinations performed were:
- Ophthalmology: bilateral amblyopia (nistagmus + foveal hypoplasia).
Strabismus control.
- Magnetic resonance imaging of the head: study of the cervicofacial region performed using TSE T1 and TSE T2 sequences in the axial plane, TSE T1 and axial TSTIR sequences coronal fat administration and TSE
Pre- and post-contrast SE T1 sequences of intracranial structures and phase-contrast venographic sequence are also obtained.
An extensive left lateral tumor was observed, extending from the temporo-occipital region to the proximal third of the neck.
Its maximum size is 17 x 12 x 5 cm in the longitudinal, anteroposterior and transverse axes.
It is a poorly defined and growing lesion.
Its surface is irregular and fistulous tracts are identified.
Its signal intensity is heterogeneous and isointense with the muscle in T1 sequences, slightly hypointense with respect to subcutaneous cellular tissue in the T2 sequence and slightly hyperintense in the TSTIR sequence.
After contrast administration, contrast enhancement is heterogeneous with areas that do not enhance in relation to necrosis or areas of abscess.
The lesion extends from the scalp in the left occipital-temporal region.
In the occipital bone disrupt the left lateral scala without the internal cortical bone.
It destroys the mastoid margin of the occipital bone, coming to contact with the proximal region of the sigmoid sinus and shows slight epidural growth in the lateral face of the posterior fossa.
It destroys the mastoid, the base of the petrous bone and the external auditory canal, reaching the anterior aspect of the petrous bone and affecting the posterior wall of the middle cranial fossa.
Infiltrates the temporalis muscle below and above.
It extends to the left masseter muscle.
Infiltrates the left parotid gland, partially respecting the deep lobe and inserts into the parapharyngeal space and contacts the vascular space.
It extends into the left submandibular space and reaches the submandibular gland medially displacing the tonsil and lateral pharyngeal mucosa, although it does not seem to appear to be pharyngeal mucosa.
It is introduced into the left mandibular fossa anteriorly displacing the condyle and horizontal branch of the mandible.
Edema of the pterygoid fossa was observed, with no clear signs of allergy.
It affects the posterior cervical space and in the skull-vertebral transition, the occipito-cutaneous muscles and the posterior segment left.
The neck is extended to the left mental cystoid.
The lesion adheres to the mastoid margin of the occipital bone, compresses the sigmoid sinus by contacting approximately 50% of its circumference and decreasing its light.
After contrast administration, enhancement of the adjacent dura mater is observed, probably due to sigmoid sinus wall injury.
Venous flow was detected in the venographic study, although it decreased in relation to the right side.
In conclusion, this is an extensive neoformation in the left lateral region of the head and neck with growth plate and ulcerated surface.
Partially destroys the left temporal and occipital bone.
Acquirement of temporary, masticator, posterior cervical, parotid, parapharyngeal and submandibular spaces on the left side of the neck and extending the mental ridge.
Check the caliber of the left sigmoid sinus.
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- head-neck CT: destruction of the entire thickness of the diploe is observed affecting the mastoid and tympanic portion of the left temporal bone.
There seems to be no occipital bone involvement.
The left mandibular condyle is displaced, although it does not show signs of tumor necrosis.
No erosion of the cervical vertebral bodies was demonstrated.
A large mass of the temporal and left laterocervical soft tissues, as described in the previous MRI study.
- Wound exudate (serials): Proteus mirabilis, Strepto group G, Provideart coagulaii Staphylococcus, Morganella morganii, Corynebacterium fragilsa negative, guiller
- schistosoma eggs in urine: not observed.
- Coprocultive: enteropathogenic bacteria are not isolated.
- An infected rotavirus and adenovirus in faeces:
- Rectal exudate: E. Coli (BLEA).
Subsequent negative serial checks.
- HIV, HBsAG, anti-HBc, HCV: negative.
A biopsy of the tumor was subsequently performed confirming the diagnosis and debridement of the infected necrotic tissue was performed.
After carrying out the studies, we present the case in the Tumor Committee and decide on treatment with radical radiotherapy and cisplatin chemotherapy for three months, after signing the informed consent form by the patient's tutors, the following scheme is performed:
Linear accelerated irradiation plan, electrons of 15 MeV, with a conformal applicator of 20 x 20 cm2, fixed technique, 5-week, 2 Gy / fraction, up to a dose of 24 Gy.
Subsequently, the first field reduction was performed with a linear accelerator, photons X of 6 MV, using 2 convergent oblique fields, consisting of multi-weekly, dosimetry in 3D and contraceptive doses up to 30 Gy.
Over-printing was also performed on left periauricular tumor, with electrons of 15 MeV, 10 x 10 cm2 conformal locator, fixed technique, 5 doses/week, 2 Gy/fraction.
With each treatment session, a dose of 6 mg of Cisplatin/m2 was administered.
The total dose achieved was 68 Gy, with good tolerance.
Since the patient manifests sustained pain, he required healing of the lesion under anesthesia in the operating room, which were initially daily and, after clinical improvement, every other day, with good evolution, and could decrease the final radiotherapy session analgesia.
She also received antibiotic treatment, initially with amikacin and amoxicillin-clavulanate, the first one after 19 days of treatment and the second one after stopping the radiotherapy.
Also, due to intestinal parasitosis caused by stercoralis, she was treated with albendazole.
At the end of cytoreductive treatment, the mass presented a more homogeneous aspect, with a smaller size than at the beginning, and surgical excision was proposed with the intention of total resection.
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Surgical act
Six months after radiotherapy, we performed surgical excision of the tumor mass, with margins of 5 cm, left functional neck dissection, left complete parotidectomy and left petrectomy.
In petrectomy, the meninge of the temporal region is reached, and the intrapetrous trunk of the ipsilateral facial nerve is dissected.
We also removed parts of the occipital muscles.
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Located in facial nerve by means of grafts of major auricular nerve, with anastomosis from the intrapetrous trunk up to 3 distal fragments frontal branches and surgical ichthyosis located anterior defects.
to the term thyroidal defect and three-dimensional tumoral exeresis, the same vein provides a large volume of tissue by means of a free flap of abdo rectus muscle (TRAM) with microvascular anastomosis of the deep inferior artery
A second vein (superficial inferior epigastric) was termino-terminally anastomosed to the superficial jugular vein.
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The donor area of the flap was repaired by biological mesh and direct closure of the skin, with some difficulty due to the thinness of the patient.
We also implanted a 1.4 g left hemiblock to correct the closure defect.
The surgery was uneventful, requiring transfusion of two concentrates of opioids during the course of the intervention.
At 48 hours, we detected an abrupt anemization of the patient with associated hypotension, left eye mydriasis and increased free flap tension, with great hardness to the tattoo and shrinkage of the flap.
An urgent CT scan showed displacement of the left temporal dura mater towards the midline.
We performed a new urgent surgical intervention, in which we observed active bleeding of the left occipital artery with retraction of it within the scalene muscles, as well as a tension hematoma under the flap.
After drainage and ligation of the bleeding vessel, we repaired the flap in its initial position and the patient went to the recovery room without further complications.
MRI performed one year after surgery showed a possible recurrence of the lesion.
The clinical examination did not show skin lesions at that time and the possible recurrence between the rest of the petrous bone and the fat of the rectus abdominis flap was considered resectable, so in the surgical specimen resection committee it was decided that
Two months after the new diagnosis, we performed a surgical approach with resection of soft tissues between the flap, the dura mater of the middle and posterior fossa and the petrous apex, sending negative intraoperative pathological specimens.
This situation was followed by petrosectomy and direct closure of the wound.
The postoperative period was uneventful except for a local hematoma that resolved with local cures, and the Ibero-Latin American Plastic Surgery - Vol.
39 - No. 4 of 2013 345 definitive pathological report remained negative for tumor.
Once this episode has been resolved, the evolution of the patient has been uneventful until now (2 years and a half after the last surgery), performing periodic reviews in our center because the family adopts the program in Spain travels.
Only a z-plasty has been necessary in a neck bridle, at the top edge of the skeletal muscle.
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In her country, the patient carries out a normal life, with full social integration and good evolution in the studies.
