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Biomedical subjects

A Huvos

Publications and source records attributed to A Huvos.

At least 19 recordsLinked to original sources

Metastatic breast carcinoma to bone disguised by osteopoikilosis.

A case of metastatic lobular carcinoma of the breast in conjunction with osteopoikilosis is described. Widespread diffuse sclerotic bone lesions were identified on radiographs in a patient with breast carcinoma. In addition computed tomography demonstrated discrete spherical areas of increased density throughout the skeleton manifest typically by osteopoikilosis. No systemic symptoms were evident, blood parameters were normal and the lesions did not demonstrate any increased uptake of technetium on bone scan. An iliac crest bone biopsy, however, revealed metastatic disease in addition to osteopoikilosis. Conventional radiological investigations may not delineate metastasis on a background of bone dysplasia.

Bone Neoplasms↗

Mucoepidermoid carcinoma: a clinicopathologic study of 80 patients with special reference to histological grading.

We sought to review our experience with salivary mucoepidermoid carcinoma (MEC) over two decades to confirm the validity and reproducibility of histologic grading and to investigate MIB-1 index as a prognosticator. Diagnosis was confirmed on 80 cases, and chart review or patient contact was achieved for 48 patients, with follow-up from 5 to 240 months (median 36 months). Immunohistochemistry with citrate antigen retrieval for MIB-1 was performed on a subset of cases. Kaplan-Meier survival curves were generated for each stage, site, and grade according to our proposed grading system. To address the issue of grading reproducibility, 20 slides were circulated among five observers, without prior discussion; slides were categorized as low-, intermediate-, or high-grade according to one's "own" criteria, and then according to the AFIP criteria proposed by Goode et al.10 Weighted kappa (kappa) estimates were obtained to describe the extent of agreement between pairs of rating. The Wilcoxon signed rank test or the Friedman test as appropriate tested variation across ratings. There was no gender predominance and a wide age range (15-86 years, median 49 years). The two most common sites were parotid and palate. All grade 1 MECs presented as Stage I tumors, and no failures were seen for this category. The local disease failure rates at 75 months for grades 2 and 3 MEC were 30% and 70%, respectively. Tumor grade, stage, and negative margin status all correlated with disease-free survival (DFS) (p = 0.0091, 0.0002, and 0.048, respectively). The MIB index was not found to be predictive of grade. Regarding the reproducibility of grading, the interobserver variation for pathologists using their "own" grading, as expressed by the kappa value, ranged from good agreement (kappa = 0.79) to poor (kappa = 0.27) (average kappa = 0.49). A somewhat better interobserver reproducibility was achieved when the pathologists utilized the standardized AFIP criteria (average kappa = 0.61, range 0.38-0.77). This greater agreement was also reflected in the Friedman test (statistical testing of intraobserver equality), which indicated significant differences in using one's own grading systems (p = 0.0001) but not in applying the AFIP "standardized" grading (p = 0.33). When one's own grading was compared with the AFIP grading, there were 100 pairs of grading "events," with 46 disagreements/100 pairs. For 98% of disagreements, the AFIP grading "downgraded" tumors. This led us to reanalyze a subset of 31 patients for DFS versus grade, for our grading schema compared with the AFIP grading. Although statistical significance was not achieved for this subset, the log rank value revealed a trend for our grading (p = 0.0993) compared with the Goode schema (p = 0.2493). This clinicopathologic analysis confirms the predictive value of tumor staging and three-tiered histologic grading. Our grading exercise confirms that there is significant grading disparity for MEC, even among experienced ENT/oral pathologists. The improved reproducibility obtained when the weighted AFIP criteria were used speaks to the need for an accepted and easily reproducible system. However, these proposed criteria have a tendency to downgrade MEC. Therefore, the addition of other criteria (such as vascular invasion, pattern of tumor infiltration [i.e., small islands and individual cells vs cohesive islands]) is necessary. We propose a modified grading schema, which enhances predictability and provides much needed reproducibility.

Adolescent↗

p53 mutations in chondrosarcoma.

Chondrosarcoma is a primary bone tumor that has several different grades and variants. We evaluated 48 chondrosarcomas for p53 overexpression and p53 mutations. p53 expression was evaluated with immunohistochemistry using monoclonal antibodies PAb421, PAb1801, and PAb240. p53 mutations were identified with single-strand conformational polymorphism (SSCP) and DNA sequencing in selected cases. Immunohistochemistry revealed nuclear staining with PAb421 and PAb1801 in the spindle cell portion of one dedifferentiated chondrosarcoma. SSCP analysis was abnormal only in the case with positive immunostaining and localized the mutation to exons 7 and 8. DNA sequence analysis identified a point mutation of G to C in codon 276, resulting in an amino acid substitution of proline for alanine. This point mutation has been reported previously in other tumors but not in chondrosarcoma. Assimilation of our results with previous studies suggests that p53 mutations are present in a minority of chondrosarcomas but when present, are in higher grade chondrosarcomas and their variants.

Bone Neoplasms↗

Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years.

We summarize our experience with 238 cases of Langerhans cell granulomatosis (LCG), 198 of whom were followed for a median period of 10.5 years. Our patients did well unless overtreated, and no deaths were attributed to the disorder itself. The disease may appear in unifocal or multifocal form, and treatment is based on this fact. Virtually all patients recovered completely except for occasional residual orthopedic problems or residual diabetes insipidus. Several of the patients underwent subsequent pregnancies without difficulty. The granulomas primarily occur in bone, but lung, skin, and lymph nodal involvement is not uncommon. Involvement of thyroid, thymus, and other sites is rare. The hallmark of the disease is the accumulation of Langerhans cells (LCs). We review the pathology of LCG by histology, electron microscopy, and immunolabeling. LCs originally were identified in squamous epithelium, but these cells are part of the widespread system of dendritic cells. The latter cells, which arise from CD34+ progenitors, are specialized and efficient antigen-presenting cells for T-cell-mediated immunity. In LCG, however, the major associated cells are not T cells, but mature eosinophils: hence the original name eosinophilic granuloma. Confusion about terminology has been based upon the scanty and rather crude pathology reports in the original literature. The term histiocytosis X was meant to cover a spectrum of three diseases--eosinophilic granuloma, Hand-Schüller-Christian disease (HSC), and Letterer-Siwe disease (LS)--but HSC and LS have no basis in pathology and hence the terms are meaningless. The term HSC has become a synonym for multifocal eosinophilic granuloma (LCG). The term LS has been used in reporting a number of benign, malignant, or unknown conditions. We prefer the term LCG to avoid confusion with the term histiocytosis X because there is evidence that the LC is not a member of the mononuclear phagocyte system and hence not a tissue macrophage, and because the use of the term "histiocyte" has become a convenience in much of the literature when reporting incompletely understood diseases.

Adolescent↗

Laryngeal oncocytic cystadenomas. Eight cases and a literature review.

OBJECTIVE: To describe an uncommon clinical entity, laryngeal oncocytic salivary adenomas. BACKGROUND: While the nomenclature of these lesions may differ depending on their histologic appearance, these tumors are all generally part of a spectrum of clinically benign cystic and papillary lesions derived from oncocytic metaplasia and hyperplasia of minor salivary ducts. DESIGN: Eight cases of laryngeal oncocytic lesions collected from two institutions. Further clinical background and follow-up data were obtained on five of eight patients. RESULTS: Patients were mostly in their seventh and eighth decades of life, and all who were questioned had smoking histories. Hoarseness was a common presenting complaint, and all patients had polypoid laryngeal masses. One patient presented with progressive upper airway obstruction, which was ultimately fatal; the laryngeal oncocytic cystadenoma was diagnosed in this case during postmortem examination. The laryngeal lesions were predominantly supraglottic. Histologically, they consisted of oncocytic metaplasia of the minor salivary ducts, cystic dilation, and papillary and microcystic hyperplasia. No recurrences were seen in those patients with follow-up (four of eight). CONCLUSIONS: Laryngeal oncocytic lesions usually present as supraglottic masses in older patients. While they are oncologically benign and nonrecurring after endoscopic removal, they may occasionally be the cause of significant upper airway obstruction.

Aged↗

The value of thallium and three-phase bone scans in the evaluation of bone and soft tissue sarcomas.

Thirty-seven patients with newly diagnosed or treated sarcomas had 47 sets of sequential thallium scans (TS) followed by three-phase bone scan (TPBS) on the same day. The diagnosis in all patients was verified by biopsy (n = 40) or long-term follow-up studies (n = 7). The sensitivity, specificity, and accuracy of TS and TPBS in detecting sarcomatous lesions was calculated: TS sensitivity was 88%, specificity 69%, and accuracy 83%; blood flow (BF) and blood pool (BP) sensitivity was 91%, specificity 54%, and accuracy 81%; delayed bone scan (DB) sensitivity was 88%, specificity 38%, and accuracy 74%. In 17 studies the flow and blood pool parts of the TPBS and TS demonstrated the soft tissue component of sarcomas, which would have been missed if only the delayed bone scan had been performed. The TS lesion to normal tissue ratio alone was not very helpful in differentiating sarcomas from benign conditions because some benign lesions are highly cellular and vascular while some malignant lesions, such as chondrosarcoma, have poor vascularity and a less cellular chondroid matrix. However, when the thallium ratio was correlated with similar ratios calculated from the BP image, it was found that if the TS lesion to normal tissue ratio exceeded the BP lesion to normal tissue ratio (12 patients), the specificity for detecting sarcomatous lesions was 100%. Nevertheless, the reverse was not true. The positive predictive value of this observation was 100% and the negative predictive value was 37%.

Adolescent↗

Osteogenic sarcoma with clinically detectable metastasis at initial presentation.

PURPOSE: Chemotherapy and surgery have improved the length of survival for patients with osteogenic sarcoma (OS) who present without metastatic disease. We reviewed our experience with patients with OS who presented with clinically detectable metastasis to determine the prognostic factors and the effects of surgery on the primary tumor and on metastatic disease. PATIENTS AND METHODS: From 1975 to 1984 we treated 62 patients who had previously untreated OS with metastasis detected at presentation. All of these patients received intensive chemotherapy that included high-dose methotrexate; doxorubicin; and bleomycin, cyclophosphamide, and dactinomycin (BCD). Selected patients also received cisplatin. The intent of surgery was resection of the primary tumor and metastatic disease. RESULTS: Survival was extremely poor; only 11% of patients survived, with a median survival of 20 months. Survival was not affected by use of preoperative chemotherapy versus immediate surgery, and did not correlate with serum lactate dehydrogenase (LDH) level, alkaline phosphatase level, or the site of the primary tumor. Survival did correlate with age, location of metastatic disease, histologic response to preoperative chemotherapy, and completeness of surgical resection of all sites of tumor. Resection of all sites of tumor identified at initial presentation was necessary for survival. CONCLUSION: OS that presents with metastatic disease has a very poor prognosis with therapy, although therapy has achieved good results for patients without metastasis detected at diagnosis. Aggressive surgical resection of tumor is necessary for survival. The use of novel therapies at initial presentation is justified with this group of patients.

Adolescent↗

Pseudomyxoma peritonei of appendiceal origin. The Memorial Sloan-Kettering Cancer Center experience.

BACKGROUND: Pseudomyxoma peritonei is a rare clinical entity in which the peritoneal surfaces and omentum are involved with diffuse gelatinous mucinous implants. It originates from ruptured mucinous tumors of the appendix or ovary. METHODS: The authors examined the experience with 34 patients with pseudomyxoma peritonei seen at Memorial Sloan-Kettering Cancer Center from 1952-1989. Of these, 17 cases were identified to be of appendiceal origin. RESULTS: All patients underwent celiotomy and cytoreduction. The median survival time from diagnosis was 75 months. It was found that long-term survival can be achieved by operation alone. When conditions do recur, chemotherapy may be valuable. CONCLUSIONS: Pseudomyxoma peritonei of appendiceal origin is a rare low-grade malignancy. Initial treatment consists of cytoreduction in an attempt to render the patient locally disease-free. Long-term survival can be obtained by operation alone, even if gross disease is present at the end of the procedure. Systemic chemotherapy should be reserved for patients with proven recurrence.

Adenocarcinoma, Mucinous↗

Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience.

PURPOSE: Adjuvant chemotherapy improves disease-free survival (DFS) for patients with osteogenic sarcoma (OS). We reviewed our experience with OS to determine prognostic factors, the role of preoperative chemotherapy and subsequent histologic response, and the role of salvage chemotherapy after poor initial response. METHODS: From 1975 to 1984, we saw 279 patients with previously untreated OS without metastasis. All patients received intensive chemotherapy and underwent surgical resection of primary tumor. Chemotherapy included high-dose methotrexate; Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH); and bleomycin, cyclophosphamide, and dactinomycin (BCD). Selected patients also received cisplatin. RESULTS: DFS was not affected by use of preoperative chemotherapy versus immediate surgery, by use of limb-sparing surgery versus amputation, age, sex, or dose intensity of chemotherapy. DFS did correlate with serum lactate dehydrogenase (LDH), alkaline phosphatase, primary tumor site, race, and histologic response to preoperative chemotherapy. There was no difference in DFS for patients with a poor histologic response who did or did not receive cisplatin, although patients who did receive cisplatin had a longer time to relapse. The 5-year DFS was 76% for patients aged less than or equal to 21 years who had extremity primary tumor and were treated with the T10 protocol. CONCLUSIONS: Intensive chemotherapy can achieve DFS for a high proportion of patients with OS. Although it is a powerful predictor of DFS, histologic response to preoperative chemotherapy cannot be assessed at diagnosis. We have not shown an ability to salvage patients with an unfavorable response. We need to increase the proportion of patients with a favorable response, identify the patients who will have an unfavorable response, and develop novel treatments to salvage poor responders.

Adolescent↗

Case report 671: Arteriovenous malformation of the right lower extremity with associated intraosseous hemangiomatosis.

Since extensive involvement of the lower extremity with arteriovenous malformations occurs often combined with the potential risk of hemorrhage, transcatheter embolization has become an attractive alternative to surgical resection [7, 9]. We describe an unusual case of extensive intraosseous hemangiomatosis, with a concomitant arteriovenous malformation that presented in a 35-year-old woman as synovitis in a knee. MR images demonstrated that the lesion was far more extensive than originally suggested and directed the subsequent biopsy and embolization. The differential diagnosis was discussed and the subject of intraosseous hemangiomatosis with associated AVM was considered in some detail.

Adult↗

Influence of the extent of resection on survival after curative treatment of gastric carcinoma. A retrospective multivariate analysis.

The therapeutic value of extensive gastric resection and regional lymphadenectomy in the curative treatment of gastric adenocarcinoma is controversial. We undertook a retrospective study of 210 patients treated with curative intent from 1960 to 1980. A multivariate survival analysis using the Cox model revealed five significant variables predictive of death from gastric cancer--two inherent pathologic factors: (1) nonpyloric site and (2) metastases in more than three lymph nodes, and three treatment factors that could often be controlled by the surgeon: (3) microscopic positive gastric resection margin, (4) inadequate lymphadenectomy, and (5) total gastrectomy. These observations reaffirm the value of wide gastric resection and adequate lymphadenectomy but argue against a general policy of elective total gastrectomy in the curative treatment of gastric carcinoma.

Adenocarcinoma↗

The role of radioactive colloids in malignant peritoneal mesotheliomas.

Malignant peritoneal mesothelioma remains a therapeutic problem. A review of the use of intraperitoneal 32P and a review of the status of therapy for this disease are presented. Six patients treated with 32P and combinations of external radiation therapy, surgery, and chemotherapy are reviewed with survival times from 5 months to 18 years. Overall survival in this disease is poor, about 44.2 months. This series suggests that with combined therapy with intraperitoneal 32P and chemotherapy, longer survivals can be achieved.

Adolescent↗

Radiation induced sarcomas of bone following therapeutic radiation.

Because of new therapeutic trends of multi-modality and the importance of late effects, we have updated our series of radiation induced bone sarcomas seen at Memorial Sloan-Kettering Cancer Center over the past four decades. A total of 37 cases of bone sarcoma arising from normal bone in the irradiated field was analyzed. The median for latent period from irradiation to diagnosis of bone sarcoma was 11 years with a minimum latent period of four years. The median radiation dose for the bone sarcoma was 6000 rad in 6 weeks with a minimum total radiation dose of 3000 rad in 3 weeks. We have found nine patients who developed bone sarcomas in the radiation field after successful treatment of Hodgkin's disease. Criteria for radiation induced bone sarcomas and the magnitude of the risk of bone sarcomas are briefly discussed.

Bone Neoplasms↗

Pelvic Ewing's sarcoma. Advances in treatment.

In a study of eighteen patients with pelvic Ewing's sarcoma who were treated with a multidisciplinary approach, chemotherapy was effective in controlling systemic spread of the tumor. Surgery coupled with improved methods of chemotherapy provided results that were statistically superior to those obtained with radiation and chemotherapy alone in control of the local pelvic lesion. A twofold increase in the survival rate was seen at a median follow-up of thirty-six months in the patients who had the resection. Our results suggest that pelvic Ewing's sarcoma is best treated by initial chemotherapy, followed by local wide marginal resection of the pelvic lesion coupled with perimeter radiation therapy and concluded with additional chemotherapy. Survival rates of patients with pelvic Ewing's sarcoma may then approach the excellent survival rates of patients with lesions in more favorable anatomical locations.

Adolescent↗

Radiation-induced soft-tissue and bone sarcoma.

From the records of Memorial Hospital of the past 50 years, 47 cases with an established diagnosis of radiation-induced sarcoma were identified and divided into two groups: the first included 20 cases of soft-tissue sarcoma arising from irradiated tissues, and the second comprised 27 cases of bone sarcoma arising from normal bones in the irradiated field. Medians for the latent periods from irradiation to diagnosis of bone and soft-tissue sarcoma were 11 and 12, years, respectively. In bone sarcomas, the latent period was longer after larger radiation doses and children appeared to be more susceptible to cancer induction than adults. Criteria for establishing the diagnosis of radiation-induced sarcoma and the magnitude of the risk of bone sarcoma are discussed.

Adolescent↗