Abstract
P 049
Pilomatrix carcinoma of the orbit
Julia Katinka Müller-Richter1, Oliver Driemel2, Urs Müller-Richter3
1Augenzentrum Würzburg; 2Klinik für Mund-, Kiefer- und Gesichtschirurgie, Universitätsklinikum Regensburg, Regensburg; 3Klinik für Mund-, Kiefer- und Plastische Gesichtschirurgie, Universitätsklinikum Würzburg, Würzburg
Background
Pilomatrix carcinoma, the malignant variant of pilomatrixoma, is a very rare, malignant, epithelial tumour with hair matrix differentiation. Head, neck and back are main localisations. It shows local aggressive growth with ulcerations and 50-60% local recurrence. Metastases (mainly bone, lung and in lymph nodes) are found in 10%. There is no gold standard for treatment. Radical excision with 1-2cm margins is recommended.
Case report
A 17-year-old female patient presented because of a swelling at the left orbital region that has been growing for the last 4 months. Active and passive opening of the eyelids was not possible because of the monstrous swelling. The nose was dislocated over midline and nasal respiration on the left side explicitly reduced.
Clinical Course
To confirm diagnosis biopsies were done. The histological examination diagnosed a low differentiated ulcerative pilomatrix carcinoma. Computed tomography showed an extraconal tumour at the left orbit, metastatic lymph nodes located preauricular, submandibular and at the mandibular angle all on the left side. Systemic metastases were ruled out. Neoadjuvant chemotherapy was started but was cancelled early by the patient.
On the next visit a tumour progression was found. To reduce tumour volume an operative resection was done. A complete removal was not achieved (pT4 pN2b (3/36) cM0 G3 R1). Adjuvant radio-chemotherapy was refused by the patient. An adjuvant radiation therapy was cancelled after two weeks by the patient. Chest pain caused the patient to come in the hospital again. X-ray examination revealed multiple osteolytic metastases in the vertebral bodies. Further examination showed peritoneal carcinosis (T4 N3 M1 G3 R1). After declining palliative radiation therapy the patient died a few days later.
Conclusion
This case of a pilomatrix carcinoma shows impressively how fast this tumour may grow and metastasize. Therefore in case of atypical pilomatrixoma the malignant type has to be ruled out.
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