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Biomedical subjects

R A Erlandson

Publications and source records attributed to R A Erlandson.

At least 19 recordsLinked to original sources
PubMed2000

Ultrastructure of submucosal glands in human anterior middle nasal turbinates.

The abundant glands situated in the lamina propria of the human anterior middle nasal turbinate were complex tubules that consist of serous, seromucous, and mucous cells, either singly or in combination. Serous granules were homogeneously dense, but could have a small lighter core. Seromucous granules had a dense rim and a large compartment of appreciably lighter density. Gradation between serous and seromucous granules made precise identification of these secretory cell types difficult. Mucous cells were of conventional morphology. The secretory tubules, which possessed a complement of myoepithelial cells, gradually transformed into ducts or the changeover was relatively sudden. The ductular portions of the tubules consisted either of tall prismatic cells or of shorter columnar cells, both of which lacked secretory granules, but had many mitochondria in their supranuclear cytoplasm. In many cases the ducts, for most of their length, consisted of secretory cells. These glands clearly participate in the elaboration of the glycoconjugate coat that serves to protect the nasal mucosa and keeps it from drying out.

Adult↗
PubMed2000

Large plaque-type blue nevus with subcutaneous cellular nodules.

Unusual or atypical melanocytic nevi can be confused with malignant melanoma. The authors present two cases of an unusual variant of blue nevus that were misdiagnosed initially as malignancy. Both lesions were asymptomatic and characterized clinically by childhood onset, with slow enlargement during adolescence and subsequent nodule formation. One lesion, which measured 24 cm in greatest dimension, was located on the anterior chest wall of a 53-year-old woman. The other lesion, which measured approximately 15 cm in greatest dimension, was located on the lateral abdominal wall of a 20-year-old man. Both lesions were characterized by a multifocal dermal and subcutaneous proliferation of fusiform and dendritic pigmented melanocytes. The histologic appearance of individual foci ranged from dermal melanocytosis to common blue nevus and cellular blue nevus. The cellular foci were located in the subcutis and involved, in one patient, the stroma of the breast. The cells were immunoreactive for S-100 protein, gp100 (HMB-45), and Melan-A (A103). Ultrastructural analysis revealed melanocytes typical of blue nevus. The woman underwent complete excision of the lesion, and the man underwent only partial excision of the lesion. On clinical follow-up of 32 and 19 months, respectively, both patients are alive and well with no evidence of recurrence or progression. Because the lesions presented clinically as large plaques and were diagnosed histologically as blue nevi with subcutaneous foci of cellular blue nevus, we term this rare variant of blue nevus large plaque-type blue nevus with subcutaneous cellular nodules. Recognition of this lesion enhances our knowledge of the morphologic spectrum of melanocytic tumors and helps to avoid confusion with malignant melanoma.

Abdominal Muscles↗
PubMed2000

Cytokeratin-positive malignant tumors with reticulum cell morphology: a subtype of fibroblastic reticulum cell neoplasm?

Cytokeratin-positive interstitial reticulum cells (CIRCs) have been described as a subset of fibroblastic reticulum cells (FBRCs) normally found in lymph nodes, the spleen, and tonsils. Although tumors derived form other reticulum (dendritic) cells, specifically follicular dendritic cells, interdigitating dendritic cells, and cytokeratin-negative FBRCs, have been well documented and are now accepted, this is not the case for tumors of CIRCs. A possible reason for this failure is the difficulty in distinguishing them from other tumors, particularly carcinoma. We report three cases of cytokeratin-positive malignant tumors with a reticulum cell morphology: two located in the mediastinum and one in the soft tissue in the proximal forearm. All cases coexpressed vimentin, and one case coexpressed smooth muscle actin and desmin, resulting in a phenotype similar to that of some normal CIRCs. Although metastatic carcinoma from an occult or regressed primary tumor cannot be excluded completely, we raise the possibility of a CIRC origin for these cases.

Actins↗
PubMed1999

Thrombotic microangiopathy as a complication of long-term therapy with gemcitabine.

Three patients with pancreatic carcinoma treated with gemcitabine for 1 year developed clinical and laboratory findings compatible with an indolent form of the hemolytic-uremic syndrome. Renal biopsy specimens in two of these patients showed the characteristic features of thrombotic microangiopathy, and a skin biopsy specimen from the third patient, who presented with livedo reticularis, showed intravascular fibrin deposition. Thrombotic microangiopathy may represent a toxic effect of long-term gemcitabine therapy.

Aged↗
PubMed1999

Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized.

"Hyalinizing spindle cell tumor with giant rosettes" is a tumor recently described by Lane et al. and thought by them possibly to represent a form of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. We report an example of this tumor on the arm of a 28-year-old woman. The ultrastructural study of the tumor confirmed the fibroblastic nature of the lesion, which subsequently metastasized to the lung. Histologically, the giant rosettes simulated palisaded granulomas. Our findings warrant classifying the tumor as a sarcoma, and we suggest the designation low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes).

Adult↗
PubMed1998

Skeletal and extraskeletal myxoid chondrosarcoma: a comparative clinicopathologic, ultrastructural, and molecular study.

BACKGROUND: Skeletal myxoid chondrosarcoma (SMC) is considered to be either a typical chondrosarcoma with prominent myxoid alterations or an altogether unique malignant cartilage tumor. Extraskeletal myxoid chondrosarcoma (EMC) is a relatively rare but well-recognized neoplasm. It was initially thought to be a low grade sarcoma of cartilage derivation and was recently found, in most cases, to contain a reciprocal t(9;22), resulting in a fusion of the EWS and CHN genes. Are SMC and EMC the same entity arising in two different locations, or are they two separate entities? To the authors' knowledge, this study represents the first systematic attempt to answer this question. METHODS: Forty consecutive cases of EMC (20 cases) and SMC (20 cases) were compared by light and electron microscopy, immunohistochemistry, and molecular analysis. The mean clinical follow-up for both groups was 55 months. Histologic criteria for SMC consisted of 95% myxoid matrix, with only minimal hyaline cartilage formation. RESULTS: The gender distribution was identical in both groups (13 males and 7 females). The mean age was 55 years for EMC patients and 45 years for SMC patients. The EMC tumors were predominantly located in the deep soft tissues of the lower extremity (60%) and buttock (20%), and the mean tumor size was 13 cm. SMC was most commonly located in the bones around the hip joint (pelvis 35%; proximal femur 20%) and shoulder (20%); the mean size was 9 cm. Histologic grade in the EMC group correlated with survival (82% of the high grade tumors metastasized). Electron microscopy performed in 8 EMC cases revealed intracisternal microtubules in 3 cases and prominent mitochondria in 5, whereas in 5 SMC cases it revealed only inconspicuous organelles. Molecular analysis for the EWS-CHN fusion RNA resulting from the t(9;22) was performed in 15 cases (9 EMC and 6 SMC) and was detected in 7 of 9 EMC cases and 0 of 6 SMC cases. In one case, the molecular structure of the EWS-CHN fusion RNA was novel. The probability of metastasis was significantly higher (P=0.004) for the EMC group than for the SMC group. CONCLUSIONS: Although similar light microscopic features are noted in EMC and SMC, fundamental differences are noted at the ultrastructural and molecular levels, suggesting that EMC and SMC represent two distinct entities in the chondrosarcoma family of tumors.

Adolescent↗
PubMed1998

Role of electron microscopy in the evaluation of soft tissue neoplasms, with emphasis on spindle cell and pleomorphic tumors.

The current significant role of transmission electron microscopy in the evaluation of soft tissue tumors when correlated with conventional histological and immunohistochemical studies is discussed for the following entities: myxofibrosarcoma, storiform-pleomorphic fibrosarcoma (malignant fibrous histiocytoma), and myofibrosarcoma; dermatofibrosarcoma protuberans; hemangiopericytoma; monophasic synovial sarcoma; extrarenal rhabdoid tumor; soft tissue perineurioma; and gastrointestinal stromal tumors, notably the so-called autonomic nerve variant.

Adult↗
PubMed1998

Presacral carcinoid tumors: report of three cases and review of the literature.

The clinical, microscopic, immunohistochemical, and ultrastructural features of three carcinoid tumors of the presacral region are reviewed. All tumors occurred in young women and did not involve the rectum. The predominant microscopic pattern was trabecular. The differential diagnosis included paraganglioma and myxopapillary ependymoma. Immunohistochemically, neuroendocrine markers and low molecular weight cytokeratins were expressed in all cases. Neurosecretory granules were identified in the single case studied by electron microscopy. One case was associated with a tailgut cyst (retrorectal cystic hamartoma). Two patients were treated with complete local excision and are free of disease 3 and 4 years after surgery. One case metastasized to both breasts and recurred locally after an incomplete excision. This report expands the already long list of sites where carcinoid tumors can arise. The frequent association of these tumors with tailgut cysts and their histologic similarities to rectal carcinoid tumors suggest that the most likely derivation of presacral carcinoid tumors is from hindgut rests.

Adult↗
PubMed1998

Neuroendocrine differentiation is a common feature of thymic carcinoma.

Immunohistochemical evidence of neuroendocrine differentiation in the form of reactivity for synaptophysin, neuron-specific enolase, and/or chromogranin was found in 11 of 19 (58%) thymic carcinomas having the typical morphologic features of that tumor type. Four of these 19 cases were studied ultrastructurally, and neuroendocrine-type cytoplasmic dense-core granules were found in two. In contrast, 84 thymomas were negative for these markers, except for a focal immunoreactivity for neuron-specific enolase in areas of medullary differentiation in half of the lymphocyte-rich tumors. The results of this study show that in the thymus, similar to most other organs, neuroendocrine differentiation is not limited to tumors with an identifiable neuroendocrine appearance in hematoxylin-eosin-stained slides, such as carcinoid tumor and small cell carcinoma, but rather that it represents a common event shared by the major types of malignant epithelial tumors of that organ.

Chromogranins↗
PubMed1998

Changes in nasal epithelium in patients with severe chronic sinusitis: a clinicopathologic and electron microscopic study.

OBJECTIVE: Defective ciliary ultrastructure and impaired mucociliary clearance play an important role in the development of respiratory disease and sinusitis. Changes in the ciliary ultrastructure of the sinonasal epithelium have been documented in patients with primary ciliary dyskinesia. However, secondary ciliary dyskinesias and epithelial cytopathologic changes have been underappreciated as a consequence of respiratory dysfunction and chronic sinusitis. STUDY DESIGN: Thirty-two patients with severe chronic sinusitis were evaluated for ciliary and epithelial abnormalities. MATERIALS AND METHODS: Fourteen patients (44%) were children who underwent full allergy, sweat, and immunologic workups. Eighteen patients (56%) were adults who had severe refractory sinusitis and had failed previous sinus surgery. All patients underwent nasal epithelium biopsies of the middle turbinate and evaluation by light and transmission electron microscopy. RESULTS: Ciliated cells were found in 23 patients (72%) with 9 patients (28%) having no cilia. Foci of normal ciliated epithelium were found in only 19% of the patients, often in epithelial invaginations. Variable numbers (usually a minor population) of cilia in 20 cases (87%) exhibited ultrastructural defects including compound cilia and microtubule and dynein arm defects. All of the patients showed variable loss of differentiated epithelial cells ranging from denuded epithelium to basal cell hyperplasia often associated with squamous metaplasia, secondary to chronic sinonasal disease. The lamina propria was often edematous with dilated capillaries, plasma cells, lymphocytes, and hyperplastic seromucous glands. CONCLUSIONS: This study demonstrates that ciliary dyskinesias are primarily the result rather than the cause of chronic sinusitis. Patients with chronic sinusitis of uncertain origin exhibit a prominent loss of differentiated epithelial cells, as well as ciliary defects, most of which are likely to be secondary to the chronic disease process. These changes slow down mucociliary clearance and lead to a vicious cycle leading to chronicity.

Adolescent↗
PubMed1997

Thymic neuroblastoma in adults: report of three cases with special emphasis on its association with the syndrome of inappropriate secretion of antidiuretic hormone.

We report three cases of neuroblastoma arising within the thymus of elderly patients. All tumors consisted of primitive neuroblasts showing focal gangliocytic differentiation within nests of neuropil. All stained for neuroendocrine markers but were negative for cytokeratins and for the MIC2 gene product. One tumor was associated with the syndrome of inappropriate secretion of antidiuretic hormone, an endocrinopathy we found in three of five case reports of thymic neuroblastoma in adults. Immunohistochemical stains confirmed production of antidiuretic hormone by this tumor. One patient died of progressive disease, one patient is disease free at 18 months, and the other patient died of unrelated causes, a spectrum that reflects the variable clinical behavior others have reported. The possible histogenesis of these purely neural tumors includes malignant transformation of a mediastinal teratoma, aberrantly located sympathetic ganglia, neuroectodermal cells native to the normal thymus, and precursors of thymic epithelial cells that have differentiated along neural lines.

Aged↗
PubMed1997

Soft-tissue perineurioma. Evidence for an abnormality of chromosome 22, criteria for diagnosis, and review of the literature.

Reported herein are two examples of soft-tissue perineurioma (STP), one arising in the maxillary sinus and the other in subcutaneous tissue of the thigh. Electron microscopy and immunohistochemistry were performed in both cases. Based on our findings and a critical review of the literature, STPs are generally small, well-circumscribed but not encapsulated tumors. Histologically, most STPs resemble fibroblastic tumors, being composed of elongated, wavy cells. The immunohistochemical reactivity for epithelial membrane antigen, the lack of reactivity for S-100 protein, and the presence of ultrastructural features of perineurial cells are typical of this tumor. To explore the possibility that STP, like the intraneural variety of perineurioma, exhibits an abnormality of chromosome 22, we performed fluorescence in situ hybridization with a probe specific for the M-bcr locus, which maps to the chromosome band 22q11. In both our tumors, a high percentage of nuclei having only one M-bcr signal (44 and 96%) was observed. Our findings indicated deletion of part or all of chromosome 22 and support the view that both soft-tissue and intraneural perineurioma are part of a spectrum of perineurial neoplasia.

Chromosome Aberrations↗
PubMed1997

Renal oncocytoma: a clinicopathologic study of 70 cases.

We reviewed 954 primary nonurothelial epithelial renal neoplasms with primary resection at Memorial Sloan-Kettering Cancer Center between the years 1980 and 1995 and classified 70 cases (7%) as renal oncocytomas. The study population was composed of 39 men and 31 women, and the mean age was 65 years (range 25 to 86 years). Fifty-six patients (80%) were asymptomatic at presentation, six (4%) had flank pain, six (4%) presented with a mass, and two (3%) had hematuria. Sixty-one were treated with total or radical nephrectomy, nine with partial nephrectomy. The right kidney was involved in 35 cases (50%), the left kidney in 32 (46%). Three cases (4%) were bilateral. Sixty-one cases (87%) were unifocal, nine (13%) multifocal. All the tumors were well circumscribed but unencapsulated. Forty-five (64%) were described as brown or red, whereas the remainder were variously described as tan to yellow. Central fibrosis or scar was described in 23 cases (33%), and gross areas of hemorrhage or cystic changes in 14 (20%). The mean size was 5.2 cm and median 5.0 cm (range 1.5 cm to 14 cm). Histologically, the tumors were characterized by a mixture of architectural patterns: compact cellular nests and acini embedded in a hyalinized, hypocellular stroma were present in 62 cases (89%), a solid nested architecture in 47 cases (67%), and a variable tubular component in 50 cases (71%). Small papillae, pseudopapillae, and intratubular epithelial tufts were seen in 19 cases (27%). Cytologically, the neoplasms also showed a mixture of cell types, the most common being the classic oncocyte, which consisted of round or polygonal cells with moderate to abundant granular, eosinophilic cytoplasm, and small round nuclei with evenly dispersed granular chromatin. Small basophilic nucleoli were visible in many of these cells in all cases. Thirty-one cases (44%) had a variable number of oncocytic cells with pyknotic nuclei and 20 (30%) contained clusters of small cells with a high nuclear/cytoplasmic ratio and dense hyperchromatic nuclei (so-called oncoblasts). Foci of tubules with clear cells embedded in a hyalinized stroma were present in six cases (9%). Cellular atypia was evident in 42 cases (60%) and was marked in 21 (30%). Eleven cases (16%) exhibited mitotic activity, albeit low. No case had atypical mitoses or necrosis. Twenty-two cases (31%) had areas of calcification within the hyalinized stroma, 12 (17%) had calcospherites, and three (4%) had osseous and myeloid metaplasia. Vascular invasion was present in three cases (4%), and invasion of perinephric fat in 14 (20%). One patient presented with liver metastasis. Fourteen cases (20%) were pT1, 42 (60%) pT2, and 14 (20%) pT3. After a mean follow-up of 58 months (range 1 to 181), 62 patients (89%) were alive with no evidence of tumor, six (9%) had died of other causes, one was alive with stable metastatic disease in the liver 58 months after diagnosis, and one died with metastatic disease to bone and liver. We conclude that renal oncocytomas have a varied morphologic appearance and their pathologic diagnosis should be based on a constellation of architectural and cytologic features. The overwhelming majority of cases behave in a benign fashion, although in rare instances they can metastasize. The presence of atypical morphologic features do not alter the excellent prognosis associated with oncocytomas and do not predict an aggressive clinical course.

Adenoma, Oxyphilic↗
PubMed1997

Primary leiomyosarcoma of bone: a clinicopathologic, immunohistochemical, and ultrastructural study of 33 patients and a literature review.

Leiomyosarcoma of bone is a rare tumor in an unusual location. Previous analysis of this entity mostly involved small numbers of cases with limited follow-up. Thirty-three patients with leiomyosarcoma of bone between 1977 and 1996 were studied, and the histologic appearance and grade were correlated with subsequent treatment and clinical behavior. To be included in this study the tumor had to be intraosseous, with other primary sites of origin clinically excluded. Also, most of the sarcomatous tissue (> or =70%) had to be of intramedullary location with only limited extraosseous extension. The patient's age at diagnosis ranged from 13 to 77 years (average 44.4). The gender distribution was equal. The long bones were preferentially affected (64%), with the lower extremity, around the knee joint, predominantly involved. Five patients (15%) developed postradiation leiomyosarcomas. The histologic analysis showed that the osseous leiomyosarcomas are most commonly of the classic type, followed by the epithelioid, myxoid, and pleomorphic variants. Immunoreactivity for smooth muscle markers (smooth muscle actin, common muscle actin, desmin) was positive in all tumors, and ultrastructural confirmation was obtained in 21% of cases. All sarcomas were histologically graded, which accurately reflected the subsequent prognosis. Seventy-five percent of the lesions were high-grade and the rest low-grade. The histologic grade of the tumors correlated with both the recurrence as well as the metastatic rates and together with the clinicopathologic stage of disease represented the cornerstone on which prudent therapy should be based.

Actins↗
PubMed1996

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↗
PubMed1996

Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity.

Follicular dendritic cell (FDC) tumor is an extremely rare malignant neoplasm with approximately 17 well-documented cases in the literature. We report 13 additional cases of this distinctive neoplasm. There were seven men and six women, with a mean age of 46.5 years (range, 27-62 years). There was involvement of cervical lymph nodes (six cases), mediastinum (three cases), axilla, tonsil, spleen, and peripancreatic soft tissues (one case each). The neoplasms were grey to tan, ranging in size from 1 to 13 cm. They were formed by oval to spindle cells with eosinophilic cytoplasm growing in sheets and fascicles, with a focal storiform pattern and whorls reminiscent of those seen in meningioma. The nuclei were oval or elongated with thin nuclear membranes, inconspicuous or small eosinophilic nucleoli, and clear or dispersed chromatin. Typically, the tumor cells were intimately admixed with small lymphocytes, with a prominent perivascular cuffing. Multinucleated tumor cells were present in seven cases. Necrosis, marked cellular atypia, high mitotic rate, and/or abnormal mitoses were present in seven cases. The tumor cells were positive for CD21 (10 of 11), CD35 (10 of 11), Ki-M4p (seven of eight) Ki-FDRC1p (six of seven), vimentin (five of nine), and S100 protein (five of nine). One case stained with actin. In situ hybridization, done in six cases, did not show Epstein-Barr virus RNA sequences. Ultrastructural examination of eight cases showed long, complex, occasionally interdigitating cytoplasmic processes joined by desmosomes. The behavior of these tumors is more akin to that of a low-grade soft tissue sarcoma than a malignant lymphoma and is characterized by local recurrences and occasional metastases. Two patients died of tumor, two were alive with recurrent or metastatic disease, eight were alive with no disease, and one was lost to follow-up.

Adult↗
PubMed1995

Establishment and characterization of a novel hepatoblastoma-derived cell line.

Hepatoblastoma is the most common pediatric liver cancer, and complete resection is required for long-term survival. There are few reported hepatoblastoma cell lines available for study. In order to develop in vitro and animal models, the authors isolated an additional cell line (HB1) from a human hepatoblastoma and here report its characteristics in culture. Cells were analyzed for growth rate, clonigenicity, and tumorigenicity, using electron microscopy. Immunocytochemistry and Northern analysis for the expression of specific surface markers and genes of interest were also performed. HB1 cells required fetal calf serum but grew well in culture (population doubling time of 2 days) and had an undifferentiated appearance under electron microscopy. Epidermal growth factor (EGF) and hydrocortisone stimulated growth with or without serum, but not autocrine growth stimulation via the epidermal growth factor receptor was detected. Proteins produced by HB1 cells under normal culture conditions included alpha-fetoprotein, cytokeratins 8 and 18, and lactate dehydrogenase (with an isozyme subunit composition similar to that of liver). HB1 cells showed chronic, high expression of c-myc and Ha-ras oncogenes but no N-myc and apparently normal RB gene expression. This cell line shows characteristics in culture consistent with malignant, hepatic epithelial cells and is apparently EGF dependent. It may provide a model system for the future development of alternative therapies.

Blotting, Northern↗