Subject(s)
Alopecia/congenital , Hair Diseases/genetics , Hair Diseases/metabolism , Hair/abnormalities , Lipase/genetics , Lipase/metabolism , Mutation, Missense , Phospholipases A1/genetics , Phospholipases A1/metabolism , Adult , Alopecia/genetics , Alopecia/metabolism , Amino Acid Sequence , Amino Acid Substitution , Base Sequence , DNA/genetics , Female , Genes, Recessive , Hair/metabolism , Humans , Hydrolysis , Models, Molecular , Molecular Sequence Data , Phospholipases A1/chemistry , Receptors, Lysophosphatidic Acid/metabolism , Sequence Homology, Amino Acid , SyndromeABSTRACT
Approximately 50% of patients with adult T-cell leukemia/lymphoma (ATLL) have skin involvement, and the smoldering, skin lesion-bearing cases are often treated with various skin-directed therapies, such as phototherapy and radiation therapy. Daily oral administration of etoposide plus prednisolone (EP) is also used for smoldering-type ATLL. However, it remains unclear whether these therapies improve patients' survival. We retrospectively analyzed the prognosis of patients with smoldering, skin lesion-bearing ATLL (n = 62), who were treated, as first therapy, with one skin-directed therapy (n = 29), oral EP alone (n = 14) or a combination of skin-directed therapy and oral EP (n = 19). Multivariate analysis revealed that the hazard ratios (HRs) for the overall survival (OS) and progression-free survival (PFS) with the combination therapy were significantly lower than those with the skin-directed therapy (HR 0.1, p = 0.001; HR 0.2, p = 0.002, respectively). These results suggest that the combination of skin-directed therapy and oral EP improves the clinical outcome of patients with smoldering, skin lesion-bearing ATLL.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Leukemia-Lymphoma, Adult T-Cell/therapy , Skin/radiation effects , Ultraviolet Therapy/methods , Administration, Oral , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Combined Modality Therapy , Drug Administration Schedule , Etoposide/administration & dosage , Etoposide/adverse effects , Fatigue/etiology , Female , Humans , Kaplan-Meier Estimate , Leukemia-Lymphoma, Adult T-Cell/pathology , Leukopenia/etiology , Male , Middle Aged , Multivariate Analysis , Outcome Assessment, Health Care/statistics & numerical data , Prednisolone/administration & dosage , Prednisolone/adverse effects , Proportional Hazards Models , Retrospective Studies , Skin/pathology , Ultraviolet Therapy/adverse effects , Vomiting/etiologyABSTRACT
A case of primary desmoplastic cutaneous leiomyosarcoma is reported. A flat and elevated tan plaque, measuring 30 mm × 20 mm, was noticed in the left back of a 74-year-old Japanese male 6 months before the resection. The biopsy specimen showed an overgrowth of desmoplastic fibrocollagenous stroma, focally admixed with a less cellular proliferation of spindle cells having mildly pleomorphic nuclei, but no mitotic figures, arranged in small clusters or appearing as individual cells. Based on these features, we interpreted it as a benign keloid-like lesion. A local resection was done, and gross examination revealed a poorly demarcated grayish tumor lesion, replacing the entire dermis and extending into the subcutis. Microscopic findings demonstrated a sparsely cellular proliferation of atypical spindle cells having cigar-shaped or multi-nucleated pleomorphic nuclei and abundant eosinophilic cytoplasm with few mitotic hot spots, arranged in interlacing bundles, alternating with scattered tumor cells within an abundant desmoplastic stroma. Immunohistochemically, these atypical cells were positive for α-smooth muscle actin, HHF-35, desmin, and caldesmon, and MIB-1 labeling index was greater than 10%. Therefore, we finally made a diagnosis of desmoplastic leiomyosarcoma as a very rare variant of cutaneous leiomyosarcoma. We should be aware that owing to its characteristic features, pathologists might misinterpret it as benign when examining only small or inadequate specimens. It is thus suggested that a large panel of antibodies including smooth muscle cell markers and MIB-1 in immunohistochemistry are useful and adjunctive diagnostic aids for recognizing malignancy, especially in diagnostically difficult cases such as ours.
Subject(s)
Antibodies, Antinuclear , Antibodies, Monoclonal , Ki-67 Antigen/metabolism , Leiomyosarcoma/pathology , Skin Neoplasms/pathology , Skin/pathology , Aged , Biomarkers/metabolism , Biopsy , Diagnosis, Differential , Humans , Leiomyosarcoma/surgery , Male , Skin/metabolism , Skin Neoplasms/surgeryABSTRACT
The patient was a 63-year-old man who underwent distal gastrectomy for advanced gastric cancer with lymph node metastasis and peritoneal dissemination. One year and eleven months after the operation,an increasing CA 19-9 concentration and metastases to the liver and peritoneum were observed. Oral TS-1 was given, but had to be discontinued because of anorexia and nausea. Chemotherapy consisting of paclitaxel (PTX) and CDDP was performed. PTX (80 mg/body) was administered weekly on day 1, 8 and 15, while CDDP (50 mg/body) was administered weekly on day 1 as one cycle. After two cycles of PTX/CDDP administration,metastases to the liver and peritoneum were not detected. The patient was treated with five courses of PTX/CDDP and survived without recurrence as of this writing. PTX/CDDP was associated with few adverse events in hospital visits, and thought to be an effective chemotherapy against recurrent gastric cancer.