Differences in actin expression between primary and recurrent facial basal cell carcinomas as a prognostic factor of local recurrence.

INTRODUCTION: Due to a relatively high recurrence rate of facial basal cell carcinoma (BCC), its morbidity is very significant. AIM: To analyse the expression of α-SMA, E-cadherin, Ber-Ep4 and MOC-31 as predictors of local recurrence in a group of patients with primary and recurrent BCCs of the face in correlation with histological and clinical data. MATERIAL AND METHODS: The study cohort included 79 patients with facial BCC (52 with primary BCC and 27 with recurrent BCC) who were treated at the Department of Cranio-Maxillofacial Surgery of the Jagiellonian University, Krakow in 1997-2009. RESULTS: Significant risk factors for local recurrence included: recurrent tumour (p = 0.001), multifocal BCC (p = 0.01), incomplete tumour excision (p = 0.02) and the aggressive infiltrating histologic subtype of BCC (p = 0.05). In the group of primary BCCs, positive expression of stromal α-SMA (p = 0.03) correlated with a statistically significant higher recurrence rate and so did positive expression of α-SMA in tumour cells of recurrent BCC (p = 0.002). In the group of primary aggressive BCC subtypes, reduced expression of MOC-31 was also associated with a higher rate of relapse (p = 0.02). CONCLUSIONS: Our findings provide information about α-SMA and MOC-31 expression in primary and recurrent BCCs. These data may contribute to the formulation of a more targeted treatment plan and follow-up strategy for patients with facial basal cell carcinoma.

Vertically transmitted HPV-dependent squamous cell carcinoma of the external auditory canal : Case report of a child.

BACKGROUND: There is much evidence that high-risk human papillomavirus (HPV) plays a causative role in a subset of head and neck squamous cell cancer (HNSCC) in adults. HPV-positive tumors behave differently even in their response to treatment and are therefore a distinct subset. Both HPV-positive and HPV-negative tumors of the head and neck region are usually in the domain of adults and cases in children are rare; thus when a 2­year-old child was diagnosed with this cancer in the external auditory canal, an in-depth assessment of the tumor was considered necessary. CASE REPORT: A 2­year-old girl was born to a HPV-positive mother who was diagnosed with cervical cancer during pregnancy. The child was delivered by caesarean section and the mother died of her cancer 7 months after delivery. After the diagnosis of locally invasive HPV-positive squamous cell cancer of the external auditory canal, the child was treated surgically, and with chemotherapy and radiotherapy. Full remission was obtained lasting up to 325 weeks since treatment was started, resulting in over 6 years of disease-free survival. CONCLUSION: This is the first case of advanced, HPV-related HNSCC in a 2­year-old child, in whom the tumor was located in the external auditory canal and who made a dramatic recovery after treatment with nonradical surgery, chemotherapy and radiotherapy. The child has currently been disease free for 6 years. This case supports the observation that HPV-related HNSCC tumors appear to respond favorably to treatment despite the patient's young age and the clinically advanced stage of the tumor.

Significance of copper determination in late onset of Wilson's disease.

BACKGROUND: Wilson's disease is an autosomal recessive disorder of copper metabolism resulting from the dysfunction of a copper transporting P-type ATPase encoded on chromosome 13. When capacity for hepatic storage is exceeded, the excess copper manifests itself in toxic action. In this article, the case of a sudden unexpected death of a 22-year-old woman, as a result of a subclinical course of Wilson's disease, is reported. METHODS/RESULTS: A woman with no past medical history of underlying liver disease was hospitalized for nine days before death because of strong hemolytic anemia of unknown origin. Intoxication by lead, mercury, cadmium, thallium, zinc, chromium, manganese, and arsenic compounds was excluded. High levels of copper in blood and urine (AAS method) were found (blood: 3.90 microg/ml, urine: 8.10 microg/ml, 12,140 microg/24 h; normal - blood: 0.88 microg/ml, urine: 0.051 microg/ml, < 51 microg/24 h). Symptomatic treatment, aimed at multi-organ failure, was applied immediately. In spite of intensive care, the patient died. Post-mortem findings indicated the presence of anasacra and ascites, hydropericardium, brain edema, orange-like coloration of internal organs, and cirrhotic liver. Histopathological examination of liver slices revealed complete micro- and medionodular cirrhotic changes, focal central necrosis of the hepatocytes and cholestasis. The copper content in liver was 89.8 mug/g (normal: 3.58 +/- 1.71 microg/g). CONCLUSIONS: Assessment of tissue copper content is essential for the diagnosis of Wilson's disease in the living or after death (in living it can be helpful in proper diagnosis, and after death it enables one to ascertain the cause of a sudden death). The copper level in the liver in Wilson's disease is about 25 times higher than in the healthy liver.

[A rare case of laryngeal leiomyoma]. / Rzadki przypadek miesniaka nablonkowego krtani.

INTRODUCTION: To report a rare case of laryngeal leiomioma. Tumors of smooth muscle origin are rare in the upper aerodigestive tract, due to the paucity of smooth muscle in the area. A review of the literature confirm that this kind of neoplasm is extremely rare in the larynx and not so many cases were described in the literature so far. We described classification, treatment and clinical course of laryngeal leiomioma. MATERIAL AND METHODS: We encountered such a tumor in a 68-year-old men who was admitted to our Hospital. Hoarseness since 2 years was a main symptom. RESULTS: The tumor was removed endoscopically and was located under left vocal cord. The patient recovered well but after 6 months was admitted again to our Hospital due to recurrence of ailments. Tracheotomy and enlarged chordectomy on the left side of larynx was performed under general anesthesia. The tumor was encapsulated and could be completely resected. No complications were observed in the postoperative course and patient was discharged from the hospital in 5th day after operation. There was no evidence of loco regional recurrence 4 years post surgery and laryngeal function was preserved. All histological pictures of the specimens from the first and second operation indicated tumor as a laryngeal leiomioma. CONCLUSION: Laryngeal leiomioma is a very infrequent neoplasm and to our knowledge, this is the 38th case of laryngeal smooth muscle tumor to be reported in the world literature.

[Castleman's disease. Case report and review of the literature]. / Choroba Castlemana--opis przypadku i przeglad literatury.

Authors present a rare case of Castleman's tumor (a hyaline-vascular type) located in the neck of 27 year old male. The tumor, sized 5 x 4 cm, was surgically removed. The follow up without any sight of recurrence. A review of literature is presented, including problems of etiology, pathogenesis, clinical presentation, diagnosis, treatment and prognosis of Castleman's disease.

Concomitant carcinoid tumor and papillary urothelial neoplasm of low malignant potential in a patient with Crohn's disease: a case report.

Patients with a long-term history of inflammatory bowel diseases show an increased risk of developing colorectal cancers. In the case of Crohn's disease, such an increased risk is also associated with other malignant tumors. The report presents a very rare case of a concomitant carcinoid tumor and papillary urothelial neoplasm of low malignant potential in a 35-year-old patient with diagnosed Crohn's disease complicated by an enterovesical fistula. The carcinoid tumor and papillary urothelial neoplasm of low malignant potential were diagnosed by postoperative histopathology. In patients with Crohn's disease, concomitant occurrence of various malignancies may be observed even earlier in life.