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Biomedical subjects

P J Vogt

Publications and source records attributed to P J Vogt.

17 recordsLinked to original sources

An unproven technique with potentially fatal outcome: provocation/neutralization in a patient with systemic mastocytosis.

OBJECTIVE: To describe the risks associated with use of an unproven technique, provocation/neutralization, in diagnosis and treatment of a putative "food allergy" in a patient with systemic mastocytosis. METHODS: A case report of a 68-year-old woman with mastocytosis is reported. The patient was interviewed, examined, and all medical records were reviewed. Photos were taken, and skin and colonic biopsies were performed. RESULTS: The patient was previously diagnosed with urticaria pigmentosa but also had significant diarrhea that was well-controlled by oral cromolyn sodium. She saw a physician who practiced provocation/neutralization and was told that food allergies were the cause of her gastrointestinal symptoms. She was placed on "neutralizing" injections of milk and wheat, but experienced flushing, palpitations, and lightheadedness with syncope upon injections into her thigh, which is a skin area highly involved by visible lesions of cutaneous mastocytosis. Later evaluation revealed increased numbers of mast cells in her colonic mucosa as well as confirmation of cutaneous mastocytosis. CONCLUSIONS: The patient's previous history of urticaria pigmentosa, orally communicated by the patient, documented in medical records, and easily visible on physical examination, was discounted by a practitioner of an alternative and unproven medical treatment, provocation/neutralization. She subsequently had potentially life-threatening reactions to "provocative" skin testing and "neutralizing" injections. Patients with systemic mastocytosis are at risk for significant mast cell mediator release during immunotherapy, conventional or alternative.

Aged↗

Angiomatoid features in fibrohistiocytic sarcomas. Immunohistochemical, ultrastructural, and clinical distinction from vascular neoplasms.

The typical example of malignant fibrous histiocytoma (MFH) or dermatofibrosarcoma protruberans (DFSP) does not require ancillary studies for diagnosis. However, hemorrhage with cystic change consisting of blood-filled spaces may closely mimic a vascular neoplasm. Eight fibrohistiocytic sarcomas exhibiting these angiomatoid features, initially mistaken for vascular neoplasms, were identified from personal consultation files and review of 157 consecutive sarcomas (1985 through 1993) at the University of California-(Davis) Medical Center. They included five MFH giant-cell-type sarcomas, two MFH angiomatoid-type sarcomas, and one DFSP. Immunohistochemical analysis of paraffin-embedded material showed vimentin diffuse positive, CD68 (KP-1) diffuse positive, and factor VIII negative in all eight sarcomas; actin HHF-45 focal positive in six, diffuse positive in one, and negative in one sarcoma; desmin focal positive in two and negative in six sarcomas; and S100 protein, cytokeratin AE1:AE3, cytokeratin 10.11, and EMA negative in all eight sarcomas. Electron microscopy of three tumors exhibited neoplastic cells with fibroblastic, myofibroblastic, and histiocytic features. Weibel-Palade bodies or neolumens diagnostic of vascular differentiation were absent. The clinical characteristics and behavior of these sarcomas reflect entities in the spectrum of fibrohistiocytic lineage (MFH subtypes and DFSP) rather than vascular neoplasms. Patients with deep, large, giant-cell-type MFHs did poorly (two of four patients died from disease at 8 and 25 months). Both patients with angiomatoid MFHs showed local recurrences from large incompletely excised head and neck lesions. One died of disease at 21 months and the other is free of disease 12 months following excision of a local metastasis to the opposite side of the neck. The patient with DFSP had an 18-cm locally recurrent scalp tumor that extended into bone. Immunohistochemical and ultrastructural confirmation of fibroblastic, myofibroblastic, and histiocytic lineage and exclusion of vascular differentiation help to establish the correct diagnosis in these fibrohistiocytic sarcomas with angiomatoid features. The clinicopathologic features of these eight cases reaffirm the practical utility of MFH and DFSP as diagnostic entities in the spectrum of fibrohistiocytic sarcomas.

Adult↗

Squamous differentiation in small-cell carcinoma of the parotid gland.

Small-cell anaplastic carcinomas comprise 1% to 2% of major salivary gland malignant tumors and demonstrate an aggressive clinical course. The initial classification of salivary small-cell anaplastic carcinoma was based on the ultrastructural identification of membrane-bound dense core granules, confirming neuroendocrine differentiation. These neuroendocrine-type small-cell carcinomas were felt to arise from neuroendocrine stem cells that migrated to the salivary gland from the neural crest. Absent neuroendocrine differentiation by ultrastructural evaluation was felt to signify origin from ductal cells. Immunohistochemical study has revised this concept because many small-cell carcinomas express at least one neuroendocrine marker, even in the absence of ultrastructural evidence of neuroendocrine differentiation. In addition, glandular differentiation both by ultrastructural and light microscopic study has been found in cases showing neuroendocrine differentiation. Ultrastructural evidence for squamous differentiation, such as desmosomes and tonofilaments, has also been recognized. These new findings have led to a revision of the old histogenetic hypothesis. All of these small-cell carcinomas are presumed to arise from a hypothetical ductal stem cell that can undergo neuroendocrine, squamous, or glandular differentiation. We report a small-cell anaplastic carcinoma of the left parotid gland in a 61-year-old man with squamous differentiation identified by light microscopy and confirmed by immunohistochemical expression of predominantly high rather than low molecular weight cytokeratins. This tumor is distinctive because it shows an abrupt transition from small-cell anaplastic carcinoma with neuroendocrine differentiation to well-differentiated squamous differentiation, which was identified readily by light microscopy. Our findings support this new hypothesis of a single multipotential stem cell by demonstrating bidirectional neuroendocrine and squamous differentiation.

Carcinoma, Small Cell↗

Serous peritonitis in adult Still's syndrome.

Serous peritonitis may be an underrecognized feature of adult Still's syndrome. An illustrative case with pathologic findings is presented. Serous peritonitis generally occurs during an acute multisystem flare of Still's syndrome. Peritoneal signs, abdominal distension, and radiographic air fluid levels in the small bowel may suggest incomplete bowel obstruction. Corticosteroids and supportive management are often effective although surgical lysis of adhesions may be required.

Abdominal Pain↗

Atypical stromal smooth muscle hyperplasia of prostate.

A prostatic lesion was resected but recurred six years later with the same atypical smooth muscle hyperplasia in the stroma and without glandular atypia. Ultrastructural study confirms the smooth muscle origin and its atypicality. Review of previous reports of leiomyosarcoma of the prostate show that the minimum criteria for malignancy are gross evidence of capsular invasion and one to two mitotic figures per ten high-power field. Cellular pleomorphism and cellularity by themselves are insufficient to diagnose sarcoma.

Aged↗

Fatal bleomycin toxicity from a low cumulative dose in a patient with renal insufficiency.

A 54-year-old man with advanced non-Hodgkin's lymphoma and chronic renal insufficiency was treated with combination chemotherapy which included bleomycin. Fatal pulmonary toxicity developed after administration of a total bleomycin dose of only 60 U. Transbronchial biopsy and autopsy demonstrated pathologic findings consistent with bleomycin-induced pulmonary fibrosis. High-dose corticosteroid therapy did not appear to alter the clinical course. Extreme caution should be exercised when administering bleomycin to patients with renal insufficiency.

Bleomycin↗

The etiology of symptoms in patients with recurrent carotid stenosis.

We performed 33 carotid endarterectomies in 29 patients for recurrent carotid stenosis. The interval between the initial and second operations ranged from six weeks to 11 years with a mean of 56 months. Three types of pathologic lesions were identified: (1) recurrent atherosclerosis (RA), (2) neointimal fibromuscular hyperplasia (NFH), and (3) lesions with elements of both RA and NFH (complex lesions). Histologic examination of early-recurring lesions (less than three years) revealed NFH in 17 patients and one complex lesion. Late-recurring lesions (three years or later) were due to atherosclerosis in eight vessels, NFH in four, and both RA and NFH in three. Focal neurologic symptoms occurred in 25 (76%) of 33 vessels, and an embolic source could be identified in 16 (64%) of 25 patients. Embolic events rather than reduced blood flow due to progressive stenosis are more frequent causes of symptoms in patients with recurrent carotid stenosis than was formerly believed.

Aged↗

Leukonychia punctata and pitted nails in alopecia areata.

A 36-year-old man with long-standing alopecia areata was noted to have both leukonychia punctata and pitted nails. Histopathologic evaluation of the leukonychia punctata revealed multiple discrete parakeratotic foci throughout the entire thickness of the nail plate, suggesting both proximal and distal matrix involvement. The finding of shallow pits lined with residual adherent parakeratotic cells suggests that the pathogenesis of nail pits in alopecia areata is similar to that of psoriasis; a result of shedding of parakeratotic cell aggregates from the nail plate.

Adult↗

Carcinosarcoma (malignant mixed tumor) of the parotid: report of a case with a pure rhabdomyosarcoma component.

BACKGROUND: Carcinosarcoma or true malignant mixed tumor of the parotid gland is extremely rare, accounting for < 1% of all salivary gland malignancies. METHODS: A 63-year-old woman presented with a 5-cm right parotid mass which was resected with a radical parotidectomy and infratemporal fossa dissection. RESULTS: The tumor contained two distinctive histologic patterns, that of a poorly differentiated ductal carcinoma and a pleomorphic rhabdomyosarcoma. Additionally, a residual focus of pleomorphic adenoma was present peripherally. Immunohistochemical and electron microscopic studies confirmed the skeletal muscle differentiation. CONCLUSIONS: The pattern of combined rhabdomyosarcoma and ductal carcinoma has not been previously reported and adds further evidence to the myoepithelial derivation of these tumors.

Carcinosarcoma↗

Fine needle aspiration cytology of pulmonary Wegener's granulomatosis with biopsy correlation. A report of three cases.

BACKGROUND: Pulmonary Wegener's granulomatosis frequently presents clinically as ill-defined nodules on chest radiographs or computed tomographic scans. With refinement of fine needle aspiration (FNA) cytology as a diagnostic tool, pathologists are sometimes asked to render this diagnosis on cytologic material rather than on the more invasive open lung biopsy. Experience is currently limited to a few case reports in the cytopathology literature. We add to this by reporting the findings from five FNA biopsies performed on three patients, with open lung biopsy correlation. CASES: The cytologic findings included a background of necrotic debris, acute and chronic inflammatory cells, multinucleate giant cells and, in one case (case 2), numerous clusters of atypical epithelioid cells mimicking carcinoma. Correlation of the respective open lung biopsy specimens with the FNA material was instructive in revealing the nature of the findings on cytologic preparations. Two cases showed classic histopathologic findings. Case 3 showed a less common presentation, pulmonary hemorrhage, which was reflected in the FNA specimen. CONCLUSION: FNA biopsy cytology is a useful alternative to open lung biopsy in the diagnosis of pulmonary Wegener's granulomatosis but requires careful correlation with the clinical and laboratory findings.

Adult↗