Squamous cell carcinoma arising on scars of epidermolysis bullosa acquisita.
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Biomedical subjects
Publications and source records attributed to T Kiyosawa.
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We report the first case of metastatic clear cell sarcoma with dramatic response to DAV treatment (DTIC + ACNU + VCR). Clear cell sarcoma of tendons and aponeuroses, or malignant melanoma of soft parts, is a rare tumour that occurs predominantly in the extremities of young adults. It tends to recur locally or metastasize and the prognosis is poor. Although the importance of surgery has been established, the role of adjuvant chemotherapy has yet to be determined. DAV should be considered as a first-line palliative treatment in disseminated disease as well as adjuvant therapy after surgery of primary clear cell sarcoma.
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A 50-year-old woman with von Recklinghausen's disease, but not Carney's complex, presented with a 1-year history of a hard subcutaneous mass on her right hip and right inguinal lymphadenopathy. Histological and immunohistochemical studies of the tumour revealed schwannian and melanocytic characteristics. Local recurrence without distant metastases was observed 5 years later. Although the diagnosis of malignant schwannoma with melanocytic differentiation, rather than neurotropic melanoma, was made for the primary tumour, based on the clinicohistopathological and ultrastructural findings, the overall clinical course in this case did not seem incompatible with malignant melanocytic schwannoma.
A 47-year-old man with an angiosarcoma of the right lower extremity was cured with mainly surgery and radiotherapy despite an inguinal metastasis. Histologically, the amputation stump of the metastatic tumour showed invasion by tumour. Radiotherapy, chemotherapy, and treatment with interleukin-2 was added. Ten years later he has had no recurrence.
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We studied the effects of stem cell factor (SCF) on human skin mast cell (HSMC) survival and the proliferation of neurofibroma (NF) cells in transplanted NF in nude mice. Small pieces of cutaneous NF from a patient with von Recklinghausen's disease were transplanted subcutaneously into nude mice. Recombinant human SCF (10 or 100 ng) was injected six or seven times around the NF transplantation sites over 11 days (i.e. every other day). The number of HSMCs was reduced in vehicle-injected NF compared to the amount present before transplantation. In contrast, NF-transplanted animals that were injected with SCF (10 or 100 ng) showed preservation of mast cell numbers in the tissue. Using computerized image analysis, mast cell size in SCF-treated NF transplants was significantly altered (larger at the 10 ng dose, and smaller at the 100 ng dose) compared with the size before transplantation or in vehicle-injected tissue. Furthermore, at the higher SCF dose (100 ng) PCNA-positive NF cells showed a significant increase. These results indicate that HSMCs in transplanted NF tissue retain their capacity to respond to SCF in vivo, and that SCF contributes to the regulation of both HSMC survival and size in cutaneous NF. In addition, activated HSMCs induced by SCF may be involved in the growth of cutaneous NF in von Recklinghausen's disease. Thus, this experimental model may be useful in the study of the cellular interactions between HSMCs and other stromal cells in cutaneous NF.
We describe a rare case of multiple, subcutaneous nocardial abscesses. A 67-year-old Japanese male with bullous pemphigoid was admitted to our hospital, and systemic prednisolone and double filtration plasmapheresis were administered. During the combination treatment, he noted a solitary subcutaneous nodule on the right upper arm. The lesions gradually increased in number and disseminated. The microbial diagnosis was thought to be a Nocardia species. Administration of combination chemotherapy with minocycline and diamino-diphenyl sulfone failed to eliminate the nodules completely, so he was treated with daily injection of amikacin sulfate into the nodules. The lesions dramatically responded to this therapy and resolved in four weeks.
We have recently observed mast cell infiltration in malignant schwannoma (MS) arising in the patient with von Recklinghausen's disease. To determine the cell to cell interactions between human skin mast cell (HSMC) and MS cell, we investigated the HSMC survival and the morphological changes when cultured with MS-derived cells isolated from the patient. Partially purified HSMCs obtained from normal adult skin and cutaneous neurofibroma by enzymatic digestion were cocultured with MS-derived cells on the coverslips. The number of HSMCs stained with crystal violet were directly counted by light microscope. HSMCs cocultured with MS-derived cell feeder layer revealed significantly increased HSMC survival compared to that with normal human skin fibroblast layer at 1 and 2 weeks. Conditioned medium of cultured MS-derived cell did not influence HSMC survival. In the morphology HSMCs cultured with MS-derived cells demonstrated spindle form in close contact with the adjacent MS-derived cells, suggesting cell to cell interactions. We failed to detect stem cell factor (SCF) mRNA in cultured MS-derived cell by RT-PCR. These results suggest that MS-derived cell is capable of supporting HSMC survival in vitro. Some factor(s) other than SCF, which is associated with direct contact between HSMCs and MS-derived cells, might relate to these observations.
Melanocytic nevi may microscopically associate with clefts or slits of the nests resembling lymphatic or vascular spaces. This unique histologic feature has been known as an artifact of injection or tissue-processing. We present a case of melanocytic nevus with a prominent vascular space-like structure. We also studied whether intralesional injection of local anesthetic could reproduce similar histologic findings. A 45-year-old Japanese female visited us with a solitary, brownish papule on the chest. Histology revealed numerous nests composed of round to oval-shaped nevus cells throughout the entire dermis. In the mid-dermis, nevus cells were lined up in a layer anastomosing and forming a vascular space-like structure. These nevus cells were uniformly stained with vimentin and S100 protein but not with factor VIII-related antigen. They were also positively immunoreactive with anti-type IV collagen and anti-fibronectin. There were no significant differences in staining intensity in the nevus cells between the solid portion and the vascular space-like structure. In the experimental study, eight melanocytic nevi were removed under local anesthesia. The local anesthetic solution was then injected into the excised nevus. Intralesional injection of a considerable volume of local anesthetic was capable of causing slits or clefts of the nests and dermal edema; however, it failed to reproduce a vascular space-like structure similar to that in the present case. These findings suggest that a vascular space-like structure in melanocytic nevus is not caused by the injection alone. Some other factor(s) may play a major role in the development of such structures in melanocytic nevus.
We recently demonstrated that cultured malignant schwannoma (MS)-derived cells can support human skin mast cell (HSMC) survival in vitro. Cultured HSMCs were spindleshaped in close contract with MS-derived cells, suggesting cell to cell interaction. To elucidate the mechanism of the enhanced HSMC survival in coculture with MS-derived cells and the cellular interactions between HSMC and MS-derived cells, we examined the immunocytochemical characteristics of MS-derived cells using immunofluorescence. Morphologically, cultured MS-derived cells were polygonal with abundant cytoplasm and resembled perineurial cells. The cultured cells immunoreacted positively with vimentin, fibronectin, laminin and collagen IV, but negatively with anti-S100 protein, anti-neuron specific enolase, and anti-neurofilament (68 kd, 145 kd, 200 kd) antibodies. MS-derived cells were distinct from Schwann cells in their lack of S100 protein and also distinguishable from endoneurial fibroblasts that produce fibronectin, but never expressed laminin or collagen IV. MS-derived cells thus possess the characteristics of perineurial cells in their general morphology and their immunocytochemical properties. Immunoreactivity for substance P (SP) and neurokinin A (NKA) was found in the cytoplasm of these cells, particularly around the nuclei. Vasoactive intestinal peptide, somatostatin, and calcitonin gene related peptide were negative. From these findings, we characterized the MS-derived cell's in vitro properties and concluded that it is similar to a perineurial cell. The extracellular matrix protein, laminin, and fibronectin expressed in the MS-derived cell might contribute to HSMC survival and morphology through cell to matrix adhesion. Neuropeptides such as SP and NKA, expressed in the MS-derived cell, might play some role in enhanced HSMC survival in vitro.
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There have been no previous concrete reports of leukemoid reactions associated with squamous cell carcinoma originating in cutaneous tissue. Here we report a case of epidermal squamous cell carcinoma of the sacral region and an associated leukemoid reaction. The tumor invaded deeply and destroyed both the sacrum and coccyx. The white blood cell count was greater than 20,000/mm3. After resection of the tumor, white blood cells transiently decreased, but did not fall under 10,000/mm3. Post-operative infection by methicillin-resistant Staphylococcus aureus and Bacteroides caccae caused sepsis and further elevation of the leukocytes to greater than 50,000/mm3. The leukemoid reaction in the case appeared to have been caused initially by direct invasion of bone by epidermal squamous cell carcinoma and later by severe infection.
We described two cases of malignant schwannoma arising in patients with von Recklinghausen's disease and examined the mast cells infiltrated into histologic sections. One of the two cases histologically revealed apparent mast cell infiltration in some areas of malignant schwannoma as well as in the benign neurofibroma. The malignant lesion demonstrated significantly increased percentages of degranulated mast cells over the benign lesion using FITC-avidin staining. In an electron microscopic study, mast cells in the malignant lesion displayed empty granules, piecemeal degranulation, and canaliculi structures suggesting activation. These findings were not observed in the benign lesion. The other patient histologically showed no mast cells in the malignant lesion, although the benign neurofibroma in the patient disclosed numerous mast cells. The first patient had neither recurrence nor distant metastasis. On the other hand, the second patient without mast cells in the histology had multiple distant metastases.
BACKGROUND: Multiple glomus tumors are extremely rare and seldom demonstrate malignant change. OBJECTIVE: To describe an exceptionally rare case of familial multiple glomus tumors involving the glans penis. METHODS: Multiple glomus tumors in a father and son were studied by light and electron microscopy. RESULTS: The clinical presentation of the two patients was similar, and atypical glomus cells were identified in both son and father. The histology of the tumor cells suggested that they were malignant, yet the clinical course has been benign. CONCLUSION: Despite their malignant histologic appearance, the behavior of familial glomus tumors is benign. A conservative approach to treatment appears warranted. The diagnosis and management of these lesions should be based on clinical behavior rather than histopathology.
By employing H-2-congenic NZB, NZW and (NZB x NZW)F1 mice with either the homozygous H-2d/H-2d, H-2z/H-2z or heterozygous H-2d/H-2z haplotype, we found that in the spleen of all the congenic strains homozygous for H-2z, there were extremely high frequencies of CD5+ B cells. These cells eventually proliferated in an oligoclonal or even monoclonal fashion, and B cell-chronic lymphocytic leukemia (B-CLL) developed in some cases. Because this feature was not observed in H-2d/H-2d homozygotes or H-2d/H-2z heterozygotes, the high CD5+ B cell frequencies are apparently controlled by the homozygosity of a locus or cluster of loci closely linked to H-2z complex of NZW strain. As the CD5+ B cell frequencies in the peritoneal cavity did not differ among the H-2-congenic strains, the frequencies of these cells in the peritoneal cavity and in the spleen appear to be at least in part under separate control. Flow cytometry and Southern blot analyses using an immunoglobulin gene JH probe revealed that the H-2z/H-2z homozygotes, there was a propagation of distinct clonal populations between the spleen and the peritoneal cavity, a finding which suggested that in the major histocompatibility complex (MHC)-related microenvironments for CD5+ B cell propagation differ between the two compartments. All our findings taken together imply that certain different but related MHC haplotypes may predispose either to B-CLL or to autoimmune disease, in close relatives.