Biomedical subjects

C M Coffin

Publications and source records attributed to C M Coffin.

At least 19 recordsLinked to original sources

PubMed1992

Cutaneous angiosarcoma as a second malignant neoplasm after peripheral primitive neuroectodermal tumor.

Second malignant neoplasms (SMN) in late childhood or young adulthood in individuals who have been successfully treated for an initial malignancy have emerged as a late effect of therapy in survivors of childhood cancer. Although radiation therapy is frequently implicated, chemotherapy with alkylating agents and antimetabolites has also been associated with SMN. Soft tissue sarcomas are among the most frequent primary malignancies complicated by a SMN and account for a majority of nonhematolymphoid SMN. We present the clinical and pathologic findings in a patient who had a peripheral neuroepithelioma (primitive neuroectodermal tumor, PNET) of the soft tissues diagnosed at 17 years of age, was treated with high-dose irradiation and multidrug chemotherapy, and developed an angiosarcoma 14 years later. This case represents an uncommon combination of mesenchymal malignancies in a young patient with an unusually favorable clinical course following the diagnosis of PNET.

Adolescent↗

PubMed1992

Benign cutaneous histiocytic tumors in childhood and adolescence, excluding Langerhans' cell proliferations. A clinicopathologic and immunohistochemical analysis.

Clinical and light-microscopic findings were reviewed in 60 benign histiocytic tumors of the skin arising in patients who were 19 years of age or younger. These lesions were placed in five categories, based on the presence or absence of Touton giant cells, the relative content of vacuolated polygonal cells and spindle cells, and the extent of stromal collagen in the proliferations. The resulting distribution included 16 cases of classic juvenile xanthogranuloma (JXG), four examples of xanthomatous JXG, 11 "transitional" JXG, 11 histiocytomas, and 18 dermatofibromas. The histological appearances of the lesions were related to the elapsed time between their clinical presentation and biopsy sampling. Intervals of 3.5, 3.5, 3.6, 7, and 13.4 months were noted for each histologic group, respectively. Hence, "early" lesions (JXG variants) showed no differences in clinical duration, but significantly longer evolutions were observed for histiocytomas and dermatofibromas. Immunohistochemical studies were conducted utilizing antibodies to several "histiocytic" markers (CD68, cathepsin B, MAC387), CD45 antigen, and S-100 protein. Cathepsin B was universally expressed by all tumors, but its intensity and scope were variable. In addition, only dermatofibromas failed to show CD68 positivity. Scattered mononuclear inflammatory cells in the lesions were CD45 reactive, whereas other cell types were not so labeled. MAC387 was absent in all cases. These findings suggest that these lesions do indeed show histiocytic differentiation, and that they are probably related to peripheral blood monocytes antigenically. Differences in the histologic appearances of these cutaneous histiocytic proliferations of childhood may simply reflect dissimilar periods of clinical growth before biopsies are performed.

Adolescent↗

PubMed1992

Atypical fibrous histiocytoma of the skin and subcutis in childhood and adolescence.

The authors have observed 15 examples of a distinctive fibrohistiocytic lesion in children and adolescents which they chose to designate as "atypical fibrous histiocytoma" (AFH). Patient ages ranged from 1 to 19 years (mean 9.3 yr.). Only two cases were encountered in the first year of life, but 7 were seen in children under the age of 10 yr. The anatomic distribution of AFH showed a tendency for a truncal origin (66%), and none was located in the skin of the face, neck, or scalp. Tumor sizes ranged from 1 to 3 cm, and one-third were 2 cm or greater in maximum dimension. Histologically, AFH was characterized by a multinodular, dermal or dermal-subcuticular proliferation of spindle cells, with tapered, cytologically bland nuclei. However, nucleocytoplasmic ratios were increased when compared with those of normal fibroblasts. Nuclear chromatin was dispersed or vesicular; nucleoli were seen in a minority of cases, but mitotic activity was regularly present. Admixed giant cells were present but infrequent, cellularity was dense, and a storiform growth pattern was consistently seen. Mean followup in this group of cases averaged 75 mo. Seven patients (47%) had tumor recurrences after initial excision; in two of these, tissue margins had been free of involvement. The authors conclude that AFH of childhood is analogous to a lesion that has previously been reported as "benign fibrous histiocytoma" in adults. Complete excision and regular postoperative surveillance are recommended for these tumors.

Adolescent↗

PubMed1992

Acute appendicitis in children: value of sonography in detecting perforation.

OBJECTIVE: We determined the sonographic features of perforating appendicitis in children in order to determine the best criteria for establishing the diagnosis. MATERIALS AND METHODS: Sonograms of the right lower quadrants of 71 children with proved appendicitis were reviewed to determine the value of sonography in distinguishing between nonperforating and perforating appendicitis. The sonographic signs evaluated included the presence or absence of an appendix, an echogenic submucosal layer, increased periappendiceal echogenicity, free or loculated periappendiceal or pelvic fluid collections, and appendicoliths. The sonographic findings were correlated with the surgical and pathologic findings. RESULTS: Forty-five patients had nonperforating appendicitis, and 26 had perforating appendicitis. A sonographically visible appendix was present in all patients with nonperforating appendicitis and in 10 (38%) of 26 patients with perforation. An echogenic submucosa was noted in 27 (60%) of 45 patients with uncomplicated appendicitis but in only three (30%) of 10 patients with a visible appendix and perforating appendicitis (p < .05). In 19 of 26 patients with perforating appendicitis, sonography showed loculated periappendiceal or pelvic fluid collections; no patient with nonperforating appendicitis had a loculated fluid collection (p < .05). No statistically significant association was found between the presence or absence of perforation and free pelvic fluid, prominent periappendiceal fat, or an appendicolith. CONCLUSION: Our results indicate that sonography can be helpful in the diagnosis of perforating appendicitis. The best predictors of perforation are absence of the echogenic submucosal layer and the presence of a loculated fluid collection.

Acute Disease↗

PubMed1991

Familial aggregation of nasopharyngeal carcinoma and other malignancies. A clinicopathologic description.

Nasopharyngeal carcinoma (NPC) occurred in five members in three generations of a white American family of Scandinavian descent. Six other family members had malignancies including malignant melanoma, malignant lymphoma, squamous cell carcinoma of the tongue, adenocarcinoma of the colon, and asynchronous bilateral in situ and invasive ductal carcinomas of the breast. There was also a history of autoimmune disorders and exposure to smoke, fumes, and chemicals in some family members. Regression analysis revealed a significant covariate risk for exposure to smoking, alcohol ingestion, dust, salted or spicy foods, and poorly ventilated conditions. According to segregation analysis, the susceptibility to nasopharyngeal carcinoma and other malignancies in this family was transmitted as an autosomal codominant characteristic. A specific histocompatibility antigen (HLA) haplotype of A1-B37-DR6 was associated with a predisposition for NPC, but no linkage was identified. Laboratory studies in selected family members did not reveal significantly elevated levels of Epstein-Barr virus antibodies or serum carcinoembryonic antigen. No specific karyotypic abnormalities were identified with peripheral blood chromosome analysis. This family was an example of apparent autosomal codominant susceptibility to NPC and other malignancies. The relationship of malignancy to the HLA haplotype of A1-B37-DR6, autoimmune disorders, and cytogenetic abnormalities was intriguing but not defined clearly.

Adult↗

PubMed1990

Cellular peripheral neural tumors (neurofibromas) in children and adolescents: a clinicopathological and immunohistochemical study.

Nine examples of a cellular peripheral neural tumor (CPNT) were identified in a review of 139 peripheral nerve sheath neoplasms in children, which included 60 neurofibromas and 16 malignant peripheral nerve sheath tumors. The mean age at diagnosis of these nine patients was 7 years, with six presenting in the first decade of life and four were noted at birth. The male:female ratio was 0.5. Topographically, the tumors were located in the extremities, 4; head and neck, 3; and trunk, 2. One or another stigmata of von Recklinghausen's neurofibromatosis (VRN) was present in four patients. After initial resection, seven children remained well, but two developed a recurrence; the histology was identical to the original tumor in one case but overt malignant transformation had occurred in the second. This case was the only tumor-related death in this series. The CPNT was a circumscribed but nonencapsulated mass measuring 1.8-7.5 cm in greatest dimension in the subcutaneous and deep soft tissues and had a compact spindle cell pattern, occasional mitoses, and minor foci of typical neurofibroma. Immunohistochemical staining revealed vimentin expression in all seven cases, Leu-7 in six, myelin basic protein and S-100 protein in five, desmin in one, and actin in none. In contrast to neurofibroma and malignant peripheral nerve sheath tumors, CPNT tended to occur earlier, either congenitally or in the first decade, and slightly more commonly in females. The anatomical distribution and pattern of immunoreactivity were similar to neurofibroma. However, the cellularity and mitotic activity of these neoplasms were sufficiently disquieting as to raise concerns about the prognosis, and in one case, the tumor behaved in an unequivocally malignant fashion. When a peripheral neural tumor with the pathologic features described in this study is encountered, wide excision and careful clinical follow-up are recommended.

Actins↗

PubMed1990

Soft tissue tumors in first year of life: a report of 190 cases.

A comprehensive review of soft tissue tumors in children and adolescents disclosed the presence of 190 neoplasms in 183 patients, which were diagnosed in infants between birth and 12 months of age; these infants represented approximately 20% of our entire pediatric soft tissue tumor series. In excess of 75% of cases were pathologically benign with the hemangioendothelioma, lymphangioma, and fibromatosis-myofibromatosis constituting the majority of cases in this category. Fibrous histiocytoma and lipoblastoma were the other two benign entities. Congenital-infantile fibrosarcoma was considered a borderline tumor because of its infrequently manifested potential for metastasis; none of the 13 cases in the present study behaved in a malignant fashion. Embryonal rhabdomyosarcoma and peripheral primitive neuroectodermal tumor were the two principal types (17 of 27 cases) of malignant soft tissue tumors. In contrast to soft tissue tumors in the first two decades, those in the first year of life were more often benign despite their cellularity and presence of mitotic activity. Fibroblastic-myofibroblastic tumors were more frequent in this young age group, whereas neurogenic and myogenic tumors were relatively more common in children older than 1 year of age. The trunk and head and neck region were the preferred topographic sites rather than the extremities, which was the case in children beyond the first year of life.

Female↗

PubMed1990

A clinicopathologic and immunohistochemical analysis of 53 cases of medulloblastoma with emphasis on synaptophysin expression.

Synaptophysin (SYN) has been identified as an integral membrane glycoprotein of presynaptic vesicles in neurons and neuroendocrine cells, and as a marker for medulloblastoma and other neuronal tumors. SYN expression was studied with a monoclonal antibody (MAb) by the immunoperoxidase technique in 53 medulloblastomas. The expression of neuron-specific enolase (NSE), Leu-7 (LEU), S-100 protein (S100), glial fibrillary acidic protein (GFAP), neurofilament protein (NF), vimentin (VIM), cytokeratin (CKER), and desmin (DES) was also assessed with antisera and MAbs. SYN reactivity was present in 94% of tumors, whereas reactivity with other markers of neuronal differentiation was also observed: NSE (100%) and LEU (83%). Regarding the intermediate filament proteins, 38% of the cases were reactive for VIM, 21% for GFAP, and 9% for DES; none expressed NF or CKER. Eight percent were reactive for S100. Among the 53 cases, the male-female ratio was 1:3; 80% of DES-positive tumors occurred in females. The mean age was 10.5 yr, (60% diagnosed in the first decade; peak age incidence between 5 and 10 yr). Five tumors were discovered in patients older than 20 yr of age. Follow-up showed that 40% of patients developed a recurrence and 47% of patients died of tumor. No statistically significant relationship was demonstrated between patterns of immunoreactivity and prognosis. We conclude that SYN is a useful marker for medulloblastomas, indicating that this tumor is a primitive neuronal-neuroblastic neoplasm. However, it is but one of several immunophenotypic markers expressed by the medulloblastoma.

Adolescent↗

PubMed1989

Potentially catastrophic bleeding disorders. Approach to diagnosis and management.

Clinical and laboratory evaluation of severe bleeding can detect the presence of an intrinsic or acquired coagulation disorder. The three most common inherited coagulation disorders are factor VIII deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease. Vitamin K deficiency, liver disease, and disseminated intravascular coagulation are the most common acquired disorders. A thorough clinical history is crucial to diagnosis. Screening tests that measure prothrombin time, partial thromboplastin time, thrombin time, and platelet count permit initial classification and guide selection of more specific tests. Results can then be used to determine appropriate therapy.

Blood Coagulation Disorders↗

PubMed1989

Immunohistochemistry in the differential diagnosis in the second-look operation for ovarian carcinomas.

The second-look laparotomy is the procedure of choice to obtain peritoneal and nodal biopsies from patients who have received a full course of chemotherapy for an ovarian malignancy. If there is pathologic evidence of persistent tumor, chemotherapy is continued. In most cases, the histopathologic interpretation is straightforward in terms of positive or negative results. However, atypical mesothelial reactions or glandular inclusions, or both, in lymph nodes are potential and real difficulties in differential diagnosis. This study evaluated a panel of immunohistochemical markers, namely carcinoembryonic antigen (CEA), Leu M1, human milk fat globule protein, cytokeratin, epithelial membrane antigen, and LN2 antibody for their usefulness in differentiating benign epithelium and mesothelium from carcinoma. Only CEA and Leu M1 were specific and sensitive, respectively, for malignant tumors in this study.

Antigens, Neoplasm↗

PubMed1989

Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases.

A review of over 900 soft tissue neoplasms in children and adolescents revealed 139 neurogenic tumors in 122 patients from newborn to 20 years of age, which had been accessioned in a 25-year period. Based upon clinicopathologic criteria, 84 (60%) tumors were regarded as unequivocally benign or borderline and the remaining 55 (40%) were malignant. The average age at diagnosis for the entire series was 10 years; the sex distribution was approximately equal; and the trunk (66, 48%) was the most frequent topographic site, followed by the head and neck (39, 29%) and extremities (31, 23%). Eighteen tumors (13%) were recognized at birth. Twenty-one patients (17%) had the stigmata of von Recklinghausen's neurofibromatosis (VRN). Neurofibromas and cellular peripheral nerve sheath tumors accounted for 43% (60 cases) of the diagnoses; these 60 neoplasms occurred in 41 patients, 12 of whom had VRN. The second largest category of neoplasms (38 cases, 28%) was the primitive neuroectodermal tumor (PNET) including the subset of "malignant small cell tumor of thoracopulmonary origin" or Askin tumor. The PNETs other than the Askin tumor (26 cases) presented mainly on the trunk (63%) and head and neck region (33%), showed a male predilection (58%) and had a mean age at diagnosis of 7 years. Askin tumors (12 cases) were, by definition, confined to the chest wall or thoracic cavity, showed a female predilection (71%), and presented in the second decade (82%, mean age 14 years). There were 16 (11%) malignant peripheral nerve sheath tumors, seven of which occurred in children with VRN. Six other categories of neurogenic tumors constituted the remaining cases. Our findings indicate that neurogenic neoplasms are an important nosologic group of soft tissue tumors in the pediatric population.

Adolescent↗

PubMed1987

Current issues in transfusion therapy. 2. Indications for use of blood components.

Many factors have stimulated a recent renewal of interest in indications for and use of blood components, including red blood cells, platelets, fresh-frozen plasma, cryoprecipitate, and granulocyte concentrates. Laboratory test results often provide helpful guidelines for the decision to use blood components. In this era of cost consciousness, the most important consideration still is to treat the patient in the most expedient and beneficial way. A conservative approach with an emphasis on high-quality medical practice is to choose and use blood components with consideration of both clinical information and laboratory findings.

Adult↗

PubMed1986

Current issues in transfusion therapy. 1. Risks of infection.

Parasitic, bacterial, and viral infections may all be associated with transfusion of whole blood and components, including packed red blood cells, platelets, fresh-frozen plasma, and cryoprecipitate. Proper collection and storage techniques, careful donor selection, and laboratory screening of donor blood for evidence of syphilis, hepatitis B, and human immunodeficiency virus (HIV) infection form the basis for prevention of transfusion-transmitted infections. Viral hepatitis, notably non-A non-B hepatitis, is the most frequent infectious risk of transfusion in the United States today. The risk of HIV infection is very low because of voluntary donor self-deferral and screening of donated blood for evidence of the infection.

Acquired Immunodeficiency Syndrome↗

PubMed1986

Choroid plexus neoplasms. Clinicopathologic and immunohistochemical studies.

Choroid plexus neoplasms account for less than 1% of all intracranial tumors, with papillomas (CPPs) more frequent than carcinomas (CPCs). Immunocytochemical characterization of these neoplasms has been limited. Glial fibrillary acidic protein (GFAP), S100 protein, and keratin have been variably demonstrated by others. Ten cases were identified at two hospitals over a 25-year period; six were children and four were adults. There were seven cases of CPP and three of CPC. Extracranial metastases occurred in one case of CPC and multiple local recurrences were common. Immunohistochemical examination was performed with polyclonal antibodies to keratin, alpha-fetoprotein (AFP), desmin, neurofilament, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), and S100 protein, and with monoclonal antibodies to vimentin, 45- to 54-kd cytokeratin (CKER), and carcinoembryonic antigen (CEA). Among the seven cases of CPP, five were positive for CKER, three for keratin, two for CEA, two for NSE, and five for S100. Three cases of CPC were positive for CEA, three for CKER, and two for keratin. With one exception, when a neoplasm was positive for CEA and S100 it was also positive for CKER. Positivity for CEA in this group was associated with a more aggressive histologic pattern and heralded a worse prognosis. S100 immunoreactivity appeared to predominate in well-differentiated neoplasms. Keratin and CKER were found in both CPP and CPC, but may be useful in the distinction from ependymomas. Statistical analysis resulted in the following classification rule: If the CEA stain is positive and the S100 stain is negative, then the tumor is malignant; otherwise, the tumor is benign.

Adolescent↗

PubMed1985

Frequency of intratubular germ cell neoplasia with invasive testicular germ cell tumors. Histologic and immunocytochemical features.

Intratubular germ cell neoplasia (ITGCN) has been regarded as the preinvasive stage of testicular germ cell tumors. We evaluated its frequency in 53 testicular germ cell tumors and determined whether immunohistochemical stains for alpha-fetoprotein, human chorionic gonadotropin, carcinoembryonic antigen, and ferritin demonstrated an advantage in its detection in comparison with hematoxylin-eosin and periodic acid-Schiff (PAS) stains. Residual seminiferous tubules were found at the periphery of the invasive neoplastic foci in 47 cases. Intratubular germ cell neoplasia was detected in several or multiple seminiferous tubules in 46 cases (98%). Exquisite localization of PAS-positive intracellular granules was present in all but one case of ITGCN. Focal immunocytochemical positivity for human chorionic gonadotropin, carcinoembryonic antigen, and ferritin was noted in 2.3% of cases. We conclude that ITGCN is present in most invasive germ cell tumors and the PAS stain is very reliable in its demonstration. Antigenic expression of the proteins that we examined is extremely limited in these primordial germ cells.

Carcinoembryonic Antigen↗

PubMed1978

The pathology of Legionnaires' disease. Fourteen fatal cases from the 1977 outbreak in Vermont.

Fourteen fatal cases from the 1977 Vermont outbreak of Legionnaires' disease have been analyzed. Serious underlying diseases were present in all patients. The only consistent lesions were in the lungs. Bronchopneumonia was present in all cases and was confluent in most. No lobe of the lung was preferentially involved and consolidation was usually bilateral. Abscesses were evident macroscopically in only two cases. Microscopically, there was an extensive alveolar infiltrate of polymorphonuclear neutrophils and macrophages. Lysis of the inflammatory cells was frequently present and was associated with an increased number of bacteria. Coagulative necrosis of lung was present in a few cases, and the possibility of a bacterial toxin must be considered. Bacteria were well stained by the Dieterle stain and appeared Gram-negative in tissue imprints from the unfixed lung.

Adolescent↗

PubMedDate not supplied

Placental vanadium in gestational diabetes mellitus.

Although many studies in animal models and in cell cultures have shown that vanadate has insulin-like effects, it has not been studied in human diabetes mellitus. In this study the levels of vanadium in human placentae from 23 pregnancies complicated by gestational diabetes mellitus were compared with 18 uncomplicated non-diabetic pregnancies closely matched for maternal age, gravidity, and gestational age. Using the unpaired Student's t-test, the mid-disc placental levels in gestational diabetes (7.62 +/- 1.29 micrograms/g dry weight) were significantly lower (p less than 0.05) than controls (8.73 +/- 1.85 micrograms/g dry weight). These findings appear to be independent of placental size and birthweight. When these data were analyzed according to treatment, the vanadium levels in insulin-treated cases (8.07 +/- 1.32 micrograms/g dry weight) were not significantly different from the matched controls (8.84 +/- 1.69 micrograms/dry weight); the levels in noninsulin treated cases (7.08 +/- 1.25 micrograms/g dry weight), however, were significantly (p less than 0.005) lower than controls (8.99 +/- 1.96 micrograms/g dry weight). It is interesting to speculate that there may be increased binding of vanadium to maternal tissues in human diabetes mellitus when insulin is deficient.

Adolescent↗

PubMedDate not supplied

Fibroblastic-myofibroblastic tumors in children and adolescents: a clinicopathologic study of 108 examples in 103 patients.

A review of over 900 soft tissue tumors in children and adolescents revealed 108 fibroblastic-myofibroblastic tumors in 103 patients from newborn to 20 years of age, which had been accessioned in a 25-year period. Based on clinicopathologic criteria, 82 (76%) were regarded as benign, 14 (13%) as borderline, and 12 (11%) as malignant. The average age at diagnosis for the entire series was 7 years with a male/female ratio of 1.8:1. The most frequent topographic site was the extremities (48, 44%), followed by the trunk (31, 29%) and the head and neck region (27, 25%). Virtually 50% (51 tumors) of cases were diagnosed during the first year of life, and 73 (71%) occurred in the first decade. The known recurrence rate was 16% (17 cases). Fibromatosis of various subtypes accounted for 95% of the histologically benign group. Infantile myofibromatosis was the most frequent form of fibromatosis, followed by aggressive (desmoid) fibromatosis (20 cases, 19%). Ninety percent of infantile myofibromatoses were diagnosed in the first year of life. In contrast, 70% of aggressive fibromatoses occurred in the second decade. Associated conditions included familial desmoid fibromatosis, Gardner syndrome, and previous surgery. The borderline category was represented by the 14 (13% of the series) congenital-infantile fibrosarcomas. All of the 14 (13%) malignant tumors were classic adult-type fibrosarcomas that occurred only in later childhood and adolescence. Fibromatosis colli, fibrous hamartoma of infancy, juvenile nasopharyngeal angiofibroma, Dupuytren-type fibromatosis, infantile digital fibromatosis, juvenile aponeurotic fibroma, unclassified fibromatoses, and fibroma of tendon or nerve sheath constituted the remaining cases.

Adolescent↗