bromochloroacetic-acid and Monosomy

Parachordoma is a very rare peripheral soft tissue tumor of unknown lineage, which has been described under other names, all of which imply a similarity to chordoma. It forms a circumscribed firm tumor, usually in deep soft tissue, with a variety of histologic patterns and cytologic features, including cords and nests of cells, some of which are vacuolated. The ultrastructure and immunophenotype indicate epithelial differentiation and parachordomas are additionally S-100 protein positive. This tumor is distinct from extraskeletal myxoid chondrosarcoma and probably from soft tissue myoepithelioma. While histologically it somewhat resembles chordoma, parachordoma has a wider range of appearances, and the two neoplasms differ in their detailed cytokeratin immunophenotype and their clinical behavior. Parachordoma is a slowly growing tumor with occasional late recurrence; cases with reported metastasis have not been histologically convincing. This commentary discusses the terminology, origin, and possible nature of this enigmatic neoplasm.

Topics: Adolescent; Adult; Arm; Buttocks; Child; Chondroma; Collagen; Fascia; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Monosomy; S100 Proteins; Soft Tissue Neoplasms; Thigh; Thorax; Trisomy

Myoepithelial cell carcinoma (MCC) of the salivary gland is a rare entity. Here, we describe the karyotype of MCC. The patient was a 53-year-old man, with a rapidly growing lesion of the palate. Despite complete surgical excision, radio- and chemotherapy, the lesion rapidly harboured local and distant metastases leading to the death of the patient, 4 months after the diagnosis. On histological and ultrastructural examination, the primary tumour and the related metastases were composed of oval and spindle cells, with features of myoepithelial cell differentiation reported in the literature. Cytogenetic analysis showed a composite karyotype in the primary tumour: 45-46,XY, +3[cp3]/ 44-45,XY, -17[cp4]/ 46,XY[5]. The lymph-node metastasis was near-triploid and showed a complex karyotype. Our cytogenetic data differ from those described in benign or slowly growing salivary gland tumours showing myoepithelial cell differentiation. It is suggested that highly aggressive tumours might follow a different pathway of malignant transformation.

Topics: Calcium-Binding Proteins; Calponins; Cell Differentiation; Chromosomes, Human, Pair 17; Cytogenetic Analysis; DNA-Binding Proteins; Fatal Outcome; Genes, Tumor Suppressor; Humans; Karyotyping; Keratins; Lymphatic Metastasis; Male; Microfilament Proteins; Middle Aged; Monosomy; Myoepithelioma; Neoplasm Metastasis; Palate, Hard; Phosphoproteins; Salivary Gland Neoplasms; Trans-Activators; Transcription Factors; Tumor Suppressor Protein p53; Tumor Suppressor Proteins

Bellini duct carcinoma (BDC) is a rare and highly aggressive renal tumor whose histogenesis is still a matter of debate although a putative origin from collecting ducts has been proposed.. A primary tumor cell culture was obtained from a BDC of a 57-year-old man who presented with a mass of the right kidney. The patient died from disease progression 18 months after diagnosis. The light and ultrastructural features were consistent with previous reports on BDC. The expression of low (Ker 18) and high (Ker 5, Ker 8, Ker 10) molecular weight keratins was studied.. The BDC tumor cells displayed strong positivity for keratins, 5, 8 and 18 but did not react with the anti-keratin 10 antibody. Northern blot analysis of total mRNA revealed expression of the c-erbB-1 oncogene unlike two conventional clear cell carcinomas of the kidney used as control. Cytogenetic analysis revealed an aneuploid karyotype: 53,XY,del(1)(p34),+iso(1q),+iso(5p),+4,+7,+8,-14,del(16)(q22). No submicroscopic deletion on p14-21 and p26 regions of the short arm of chromosome 3 was detected on Southern blot analysis.. The absence of structural changes in the short arm of chromosome 3 (usually present in hereditary and sporadic renal cell carcinomas) in the presence of chromosomal abnormalities observed in malignant lesions of urothelial origin confers to BDC a unique genetic profile among papillary tumors of the kidney.

Topics: Carcinoma, Papillary; Chromosome Deletion; Chromosomes, Human, Pair 1; Chromosomes, Human, Pair 3; Chromosomes, Human, Pair 7; ErbB Receptors; Humans; Karyotyping; Keratins; Kidney Neoplasms; Kidney Tubules, Collecting; Male; Middle Aged; Monosomy; Trisomy