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Biomedical subjects

L P Dehner

Publications and source records attributed to L P Dehner.

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

renal tumors and tumor-like lesions in pediatric patients.

Renal enlargement presenting as an abdominal mass(es) is attended by a lengthly differential diagnosis of non-neoplastic and neoplastic lesions with a range in serious connotations and consequences. Simple compensatory hypertrophy and unilateral multicystic dysplasia are relatively innocuous and easily recognized with appropriate imaging studies; they are also related in the sense that the normal contralateral kidney hypertrophies in the absence of a non-functioning dysplastic kidney. Bilateral nephromegaly in a neonate is generally a sign of autosomal recessive polycystic kidney disease or multicystic dysplasia secondary to distal obstructive uropathy. Primary neoplasms of kidney in the pediatric population in the past were traditionally classified as Wilms' tumors, but that erroneous practice has been eliminated with the recognition of several distinctive neoplasms in addition to classic Wilms' tumor. Separating a typical Wilms' tumor from mesoblastic nephroma, clear cell sarcoma of the kidney and the malignant rhabdoid tumor, for treatment and prognostic purposes, has become the accepted norm in the past 12-13 years. Another important advance at the cellular level is the recognition of a deletion in the short arm of chromosome 11 in the cultured cells of Wilms' tumor and in the germ cell line in certain clinical settings of Wilms' tumors. A dramatic expansion in the understanding and management of childhood renal neoplasms has occurred through the multimodality approach of laboratory investigation and applied clinical research.

Carcinoma, Renal Cell↗
PubMed1992

Postoperative radiotherapy for malignant fibrous histiocytoma.

Between 1974 and 1989, 49 patients with histologically confirmed malignant fibrous histiocytoma received postoperative radiotherapy at the Mallinckrodt Institute of Radiology for primary (41) or recurrent (8) disease. Median age of the patients was 63 years, and the median follow-up period was 41 months. Patients were grouped according to the 1988 AJC staging classification: stage IA (one patient), stage IIA (4 patients), stage IIB (9 patients), stage IIIA (15 patients), stage IIIB (18 patients), and stage IVA (2 patients). Eight tumors (16%) were in the pelvis, 8 (16%) in the trunk, 4 (8%) in the head and neck, and 29 (60%) in the extremities. Primary surgical procedures included incisional biopsy (4 patients), excisional biopsy (19), narrow margin excision (14), wide local excision (9), and removal of the entire compartment (3). Based on pathology reports, the margins of resection were classified as positive in 23 (5 gross, 18 microscopic), 5 close, 11 negative, and 10 unknown. Patients were irradiated with shrinking field technique; the median radiation dose was 6000 cGy, with more than 95% of patients receiving at least 4500 cGy. In addition, seven patients received postoperative chemotherapy. The 5-year overall survival rate was 62%, disease-free survival 64%, local control 68%, and freedom-from-distant metastasis 85%. Thirteen patients had local recurrences, with greater than 75% recurring within 3 years. Sites of local recurrence were as follows: trunk (3), pelvis (3), lower extremities (4), and head and neck (3). There appears to be a correlation of local failure with positive surgical margin: of 23 patients with positive margins, 9 (39%) had local recurrences, whereas 1 of 11 patients (9%) with negative margins had local recurrence. Three of 13 patients with persistent or recurrent disease were salvaged by additional treatment, rendering ultimate local control in 80% (39/49). Thirty-four of 36 patients with local control obtained good to excellent function. Two patients were found to have grade 3 complications: 1 patient had edema of the extremity, and the other developed necrotic skin ulcer that was successfully treated with hyperbaric oxygen. Five patients developed distant metastases, with 80% occurring within 2 years. In summary, adequate but conservative surgery with postoperative radiotherapy for malignant fibrous histiocytoma can achieve local tumor control as well as preservation of functional limbs with acceptable morbidity in a large proportion of patients.

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

Pilomatrical neoplasms in children and young adults.

Pilomatrixoma, a neoplasm of hair germ matrix origin, is one of the most common cutaneous appendage tumors in patients 20 years of age or younger. Our review of cutaneous adnexal tumors in the first 2 decades of life showed that pilomatric lesions accounted for approximately 75% of cases. We examined 76 tumors from 69 patients between the ages of 8 months and 19 years (average age, 8 years). The female-to-male ratio was 3:1. We noted a predilection for the head and neck region, as has also been reported by other investigators. Sixty-nine tumors were examples of typical or classic pilomatrixoma; four of them showed the unusual histologic feature of transepidermal elimination or perforation. None of the tumors in the typical category recurred during a follow-up period that averaged 8.7 years. Seven of the lesions with atypical histologic features were examples of aggressive pilomatrixoma (three cases) and pilomatrical carcinoma (four cases). The average age of these seven patients was similar to that of the overall group. The pilomatrical carcinomas were characterized by invasive nests of tumor cells with irregular borders, large vesicular nuclei, prominent nucleoli, multiple mitotic figures, and focal necrosis. A desmoplastic stroma surrounded the infiltrating nests of tumor. Two of the four pilomatrical carcinomas recurred 2 and 6 months, respectively, after initial excision. The aggressive pilomatrixomas had a similar infiltrative growth pattern, but they did not exhibit the same degree of worrisome cytologic features. Single cell necrosis, identifiable mitotic figures, and prominent nucleoli differentiated the atypical from the typical pilomatrixomas. One of the three aggressive pilomatrixomas recurred after 2 months.(ABSTRACT TRUNCATED AT 250 WORDS)

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

Peripheral primitive neuroectodermal tumors. A flow cytometric analysis with immunohistochemical and ultrastructural observations.

Flow cytometry of classical neuroblastoma has provided provocative evidence that cell cycle and ploidy analysis generate prognostically useful information. To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. An acceptable histogram was obtained in 16 cases. Of these, nine neoplasms were diploid and seven contained aneuploid DNA. Among patients with diploid lesions, four were free of disease, whereas three had persistent or recurrent disease, and two had died of tumor. Among patients with aneuploid neoplasms, four were free of disease, one had recurrence, and two had died. There was no apparent correlation between immunophenotype and proliferative activity with the clinical outcome. Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system.

Aneuploidy↗
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↗
PubMed1991

Malignant mixed müllerian tumors: an immunohistochemical study of 47 cases, with histogenetic considerations and clinical correlation.

Forty-seven cases of malignant mixed müllerian tumors were reviewed histologically and studied immunohistochemically with three major objectives: to analyze the histogenetic relationship between the carcinomatous and sarcomatous components of these neoplasms, to ascertain the practical role of immunohistochemical studies in diagnosis and classification, and to determine the prognostic significance of immunohistochemically verified rhabdomyoblastic and neuroendocrine differentiation. Epithelial differentiation (cytokeratin and/or epithelial membrane antigen expression) was confirmed in all carcinomatous components; within the sarcomatous areas, it was identified among individual cells (60% of cases) and within poorly formed clusters of cells (57% of cases). There was a statistically significant tendency for concordant expression of alpha-1-antichymotrypsin, Leu-M1, S-100, Leu-7, and neuron-specific enolase between the carcinomatous and sarcomatous components of individual cases. These two findings provide evidence of common origin for the sarcomatous and carcinomatous components of these neoplasms. Histologic review of metastases in 21 cases revealed a biphasic composition in the majority of metastatic lesions (62%), another feature that further supports a common origin for the two components. From a practical standpoint, immunohistochemistry may be helpful in accentuating the biphasic pattern of these neoplasms and in verifying the presence of rhabdomyoblastic differentiation. In most cases, however, careful morphologic examination and thorough sampling will suffice for correct diagnosis and subclassification. The presence of heterologous, rhabdomyoblastic, or neuroendocrine differentiation did not have a statistically significant influence on survival; the last of these was associated with a tendency for a more rapidly fatal course.

Actins↗
PubMed1990

Germ cell neoplasms of head and neck soft tissues: a pathologic spectrum of teratomatous and endodermal sinus tumors.

Germ-cell neoplasms, in particular teratomas with immature and mature somatic type tissues, are some of the most commonly found tumors in children. Approximately 5% of these neoplasms appear in one of several extracranial sites in the head and neck region. This study reports the clinical, pathologic and immunohistochemical findings in six germ-cell neoplasms occurring in the neck and facial areas. A mass was recognized at birth in five children, and the sixth patient was 2 1/2 years old at diagnosis. Four of the six neoplasms contained one or another element of endodermal sinus tumor; two of these had a mixed pattern of endodermal sinus tumor and teratoma. The other two cases were purely teratomas. The serum alpha-fetoprotein was known to be elevated in three children whose tumors had endodermal sinus elements; it returned to normal level in two of the children, but remained high in the one fatal case. Placental alkaline phosphatase and alpha-fetoprotein were demonstrated immunohistochemically in two of the three cases, with available tissue containing endodermal sinus tumor. Teratomatous metastases in ipsilateral cervical lymph nodes were found in one patient with a pure teratoma; that patient is disease-free one year after surgery. Only nine previous examples of endodermal sinus tumor have been reported in the head and neck region, exclusive of the central nervous system. There is one other case in the literature of a congenital cervicothyroidal teratoma with metastatic disease. These six neoplasms illustrate the clinical and pathologic spectrum in this nosologically homogeneous, but morphologically diverse, category of tumors.

Alkaline Phosphatase↗
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↗
PubMed1979

Renal cell carcinoma occurring with contralateral adrenal metastasis: a clinical and pathological trap.

Because renal cell carcinoma occasionally occurs in manifestations referable to a single metastatic lesion, there is a risk that a metastasis will be mistaken for the primary tumor. Histologic examination can compound the confusion, particularly when a clear cell carcinoma of the kidney metastasizes to an organ, such as lung, liver, adrenal, or skin, where clear cell tumors may occur as primary lesions. Although advanced renal cell carcinoma not infrequently involves the adrenal gland, the clinical and pathologic setting establishes the tumor in the adrenal as a metastasis. Two patients are described to illustrate what is to our knowledge the heretofore undescribed occurrence of renal cell carcinoma as a metastasis to the contralateral adrenal gland.

Adenocarcinoma↗
PubMed1979

Malignant melanotic neuroectodermal tumor of infancy: a clinical, pathologic, ultrastructural and tissue culture study.

The melanotic neuroectodermal tumor of infancy is an uncommon neoplasm typically of early childhood which has a predilection for the head and neck region, particularly the maxilla. Except for one previous example in the literature, this tumor has consistently behaved in a benign fashion. This study documents the clinical course and pathologic findings of a tumor which began in the maxilla of a 4-month-old boy, followed by a local recurrence, metastasis to a cervical lymph node and finally, widespread dissemination and death at 18 months, 24 months and 38 months, respectively. The tumor was initially composed of nests consisting of melanin-containing cells and small dark cells. An elevated vanillylmandelic acid level was recorded during the course of the disease. At autopsy, the tumor in lymph nodes, liver, bone and soft tissues had a monotonous pattern of small dark cells similar to a conventional neuroblastoma. Previous ultrastructural studies indicate that the melanotic neuroectodermal tumor of infancy is composed of melanocytes and neuroblast-like cells. Our case provided the unique opportunity to examine in sequence the ultrastructural and in vitro characteristics of a recurring and eventually metastasizing melanotic neuroectodermal tumor. Although the neuroblast-like cells were initially difficult to identify by electron microscopy, a melanin-producing cell line and a separate nonpigmented cell line were successfully isolated from various tumor explants. Various stages of melanosome development were identified in the pigmented cells from the local recurrences and in vitro. Dibutyryl cAMP accentuated the formation of pigment and dendritic development in the melanocytes and dendrites only in the small nonpigmented cells. Electron dense granules were observed in the cultured smaller cells and also in the lymph node and soft tissue metastases. Tyrosine hydroxylase activity was demonstrated in the neuroblast-like cells. In the final biopsy and autopsy material, only the neuroblast-like cells remained and the tumor resembled a conventional neuroblastoma.

Culture Techniques↗