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J Rosai

Publications and source records attributed to J Rosai.

At least 55 records · Page 3Linked to original sources
PubMed2000

Small cell neuroendocrine carcinoma with skeletal muscle differentiation: report of three cases.

Three cases of neuroendocrine carcinoma showing skeletal muscle differentiation are presented. The tumors were located in the skin and subcutaneous tissue, the urinary bladder, and the nasal cavity respectively, and were composed by two cell types admixed intimately with each other. One cell type had features identical to those seen in conventional small cell neuroendocrine carcinoma, including scanty cytoplasm, round nuclei with fine granular chromatin, immunohistochemical reactivity for neuron-specific enolase, chromogranin and cytokeratins, and electron-dense granules on ultrastructural examination. The second cell type was either plasmacytoid or elongated and straplike, with abundant eosinophilic cytoplasm and irregular nuclei with prominent nucleoli. These cells showed immunohistochemical positivity for desmin, sarcomeric actin, myoglobin, and myogenin. They also exhibited ultrastructural evidence of rhabdomyoblastic differentiation in the form of contractile filaments with abortive Z-band formation. An origin from a cell capable of dual differentiation toward neuroendocrine and rhabdomyoblastic elements is postulated for these tumors.

Aged↗
PubMed2000

Xanthogranulomas with inconspicuous foam cells and giant cells mimicking malignant melanoma: a clinical, histologic, and immunohistochemical study of three cases.

Histiocytic proliferations can mimic melanocytic tumors and vice versa. The authors describe the clinical, histologic, and immunohistochemical findings of three predominantly mononuclear xanthogranulomas that were misdiagnosed as malignant melanoma by experienced pathologists. All lesions occurred in male patients ranging in age from 14 to 75 years. The tumors presented as dermal nodules, two of which were surrounded by an epidermal collarette and were ulcerated focally. The tumors were composed of a mixed population of large epithelioid and plump spindle cells with pink or pale cytoplasm arranged in nests and short fascicles. Occasional mononuclear cells had cytoplasmic vacuolar changes, but none had well-developed foamy cytoplasm. Rare, multinucleated giant cells were present, but they were not of the Touton type. Mitotic figures were found in all lesions. Immunohistochemically, most tumor cells (80%-90%) were strongly positive for CD68 and a minority of cells (10%-15%), located typically at the periphery of the tumor, was positive for factor XIIIa. Two tumors contained rare cells positive for S-100 protein (5% of tumor cells or less). All tumors were completely negative for tyrosinase (T311), gp100 (HMB-45), and Melan-A (A103). Giant and foam cell-poor variants of juvenile xanthogranuloma have been reported previously, mainly in young children. Their occurrence in adolescents and adults is underrecognized. Knowledge of this variant is important to avoid misdiagnosing a benign tumor as malignant melanoma.

Adolescent↗
PubMed2000

Prognostic value of [18F]fluorodeoxyglucose positron emission tomographic scanning in patients with thyroid cancer.

Poorly differentiated thyroid cancer lesions often lose the ability to concentrate radioactive [131I]iodine (RAI) and exhibit increased metabolic activity, as evidenced by enhanced glucose uptake. We incorporated [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) scanning into the routine follow-up of a cohort of thyroid cancer patients undergoing annual evaluations. One hundred and twenty-five patients who had previous thyroidectomies were included. They had diagnostic RAI whole body scans, serum thyroglobulin measurements, and additional imaging studies as clinically indicated. During 41 months of follow-up, 14 patients died. Univariate analysis demonstrated that survival was reduced in those with age over 45 yr, distant metastases, PET positivity, high rates of FDG uptake, and high volume of the FDG-avid disease (>125 mL). Survival did not correlate with gender, RAI uptake, initial histology, or grade. Multivariate analysis demonstrated that the single strongest predictor of survival was the volume of FDG-avid disease. The 3-yr survival probability of patients with FDG volumes of 125 mL or less was 0.96 (95% confidence interval, 0.91, 1.0) compared with 0.18 (95% confidence interval, 0.04, 0.85) in patients with FDG volume greater than 125 mL. Only 1 death (of leukemia) occurred in the PET-negative group (n = 66). Of the 10 patients with distant metastases and negative PET scans, all were alive and well. Patients over 45 yr with distant metastases that concentrate FDG are at the highest risk. Once distant metastases are discovered in patients with differentiated thyroid carcinoma, FDG-PET can identify high and low risk subsets. Subjects with a FDG volume greater than 125 mL have significantly reduced short term survival.

Adult↗
PubMed2000

Bone metastases from thyroid carcinoma: a histopathologic study with clinical correlates.

CONTEXT: Only limited information exists on the pathologic aspects of thyroid carcinomas with bone metastases, most large studies having concentrated mainly on their clinical features. OBJECTIVE: To study in detail the morphologic features of thyroid carcinomas with skeletal metastases. DESIGN: Seventy-nine cases of thyroid carcinoma with bone metastases treated at Memorial Sloan-Kettering Cancer Center, New York, NY, between 1964 and 1998 were investigated, with emphasis on the pathology of the primary and/or metastatic tumors and comparison of the morphologic features of the tumors at both the sites, wherever possible. The tumors were also compared for various clinical parameters. RESULTS: The cohort consisted of 22 papillary, 17 follicular, 16 insular, 10 anaplastic, 9 Hürthle cell, and 5 medullary carcinomas. Of these cases, 68% had poorly differentiated or undifferentiated features in the primary and/or metastatic tumors. The metastatic tumors were better differentiated than the primary in one third of the cases (6 of 18). Only one case showed a less differentiated metastasis. The overall 5- and 10-year survival probabilities after the bone metastases were 29% and 13%, respectively (Kaplan-Meier method). Although both the tumor type and differentiation seemed to affect survivals after bone metastasis (P =.007 and.012, respectively) (log-rank test), this was primarily due to the much worse prognosis in the cases of anaplastic and medullary carcinoma. Cases of Hürthle cell carcinoma showed the longest median survival. There was no significant difference in survival among patients up to or older than 45 years at the time of metastases (P =.31). CONCLUSIONS: Most thyroid carcinomas with bone metastases are of papillary type, and most have poorly differentiated or undifferentiated features. The influence of the microscopic tumor type and tumor differentiation on survival after bone metastasis primarily appears to be due to the much worse prognosis among anaplastic and medullary carcinomas. Age at diagnosis of bone metastases does not influence survivals.

Adenocarcinoma↗
PubMed1999

p27 and Rb are on overlapping pathways suppressing tumorigenesis in mice.

The commitment of cells to replicate and divide correlates with the activation of cyclin-dependent kinases and the inactivation of Rb, the product of the retinoblastoma tumor suppressor gene. Rb is a target of the cyclin-dependent kinases and, when phosphorylated, is inactivated. Biochemical studies exploring the nature of the relationship between cyclin-dependent kinase inhibitors and Rb have supported the hypothesis that these proteins are on a linear pathway regulating commitment. We have been able to study this relationship by genetic means by examining the phenotype of Rb+/-p27-/- mice. Tumors arise from the intermediate lobe cells of the pituitary gland in p27-/- mice, as well as in Rb+/- mice after loss of the remaining wild-type allele of Rb. Using these mouse models, we examined the genetic interaction between Rb and p27. We found that the development of pituitary tumors in Rb+/- mice correlated with a reduction in p27 mRNA and protein expression. To determine whether the loss of p27 was an indirect consequence of tumor formation or a contributing factor to the development of this tumor, we analyzed the phenotype of Rb+/-p27-/- mice. We found that these mice developed pituitary adenocarcinoma with loss of the remaining wild-type allele of Rb and a high-grade thyroid C cell carcinoma that was more aggressive than the disease in either Rb+/- or p27-/- mice. Importantly, we detected both pituitary and thyroid tumors earlier in the Rb+/-p27-/- mice. We therefore propose that Rb and p27 cooperate to suppress tumor development by integrating different regulatory signals.

Aging↗
PubMed1999

Correlation between clinicopathological features and karyotype in spindle cell sarcomas. A report of 130 cases from the CHAMP study group.

Soft-tissue tumors have proved to be a fruitful area for the identification of reproducible cytogenetic aberrations, especially among pediatric round-cell sarcomas and lipomatous tumors. Thus far, however, data regarding sarcomas of monomorphic spindle cell type have been limited and somewhat disappointing, with the notable exception of synovial sarcoma. As part of an ongoing international collaborative study, 130 karyotyped spindle-cell sarcomas were reviewed and classified histologically, without knowledge of the clinical and karyotypic data, with the aim of identifying objective correlations between morphology, karyotype, and clinical parameters. Clonal chromosomal abnormalities were identified in 82 cases studied (63%), but only in the group of synovial sarcomas was there clear correlation between the cytogenetic findings, in the form of a consistent t(X;18)(p11;q11), and morphology. Among leiomyosarcomas (41 cases) and malignant peripheral nerve sheath tumors (MPNSTs; 27 cases) as well as in individual examples of rarer entities, there was a general tendency for karyotypic complexity associated with frequent loss or rearrangement of chromosome arms 1p, 10p, 11q, 12q, 17p, and 22q. Rearrangements of 17q (the region of the NF1 gene) were seen in 9/27 (33%) of MPNSTs. Among nine cases of solitary fibrous tumor (in which previous cytogenetic data are very limited) no consistent aberrations were identified. We conclude that, with the exception of synovial sarcoma, most spindle-cell sarcomas share with pleomorphic sarcomas the tendency for karyotypic complexity. There was no indication (in most of these lesions) that detectable cytogenetic aberrations could either facilitate their diagnosis or help to determine prognosis. There is a clear need to further study and understand the significance of multiple chromosomal abnormalities in this group of mesenchymal neoplasms with the particular goal of determining their role in the process of tumor development.

Adolescent↗
PubMed1999

Hobnail hemangioma: a pseudomalignant vascular lesion with a reappraisal of targetoid hemosiderotic hemangioma.

The clinicopathologic features of 15 cutanous hemangiomas having a distinctive and frequently pseudomalignant morphologic appearance are presented. There were 5 male and 9 female patients, whose ages at diagnosis ranged from 11 to 58 years (median 30.5). An angiomatous/pigmented, nontargetoid, flat, or exophytic lesion of variable duration was the main presenting sign. The tumor sizes ranged from 0.4 cm to 2 cm (median 1 cm). The locations included the lower limb, particularly the thigh (8); the trunk, including the shoulder area (4); the head (1); the gingiva (1); and the tongue (1). One patient had two lesions; none had a concomitant vascular anomaly or was suspected to have HIV infection. Treatment consisted of excisional biopsy in all cases. Follow-up information on 10 patients (range 4-66 months; median 13 months) showed no recurrence. On microscopic examination, the lesion showed a biphasic pattern characterized by the presence of well-formed, dilated, vascular channels in superficial dermis and a collagen-dissecting, pseudoangiosarcomatous pattern as the lesion infiltrated deeper into the dermis. The lining endothelium consistently showed distinctive hobnail cytomorphology; although there were endoluminal stromal papillae, there was no endothelial multilayering or tufting. Cytologic atypia was minimal or absent, and there were no mitoses. In 3 cases, the morphologic features were reminiscent of retiform hemangioendothelioma. Immunohistochemistry performed in 8 cases showed variable reactivity of endothelial cells with CD31, CD34, Factor VIII-related antigen, and Ulex europaeus agglutinin-1 in all cases; smooth muscle actin-positive pericytes were observed focally around some of the abnormal vascular spaces. The above-described hemangiomatous lesions share many features with so-called targetoid hemosiderotic hemangioma (a clinically descriptive term), but show a variable, often minimal, amount of hemosiderin deposition. The histologically descriptive term hobnail hemangioma is proposed to designate these lesions. Hobnail hemangioma should be distinguished from well-differentiated angiosarcoma, patch-stage Kaposi's sarcoma, and retiform hemangioendothelioma, with which it may be confused.

Adolescent↗
PubMed1999

Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report.

We report an unusual case of multifocal leiomyosarcoma involving the thyroid gland, liver, and right lung in a child with congenital immunodeficiency disease. The smooth muscle nature of these neoplasms was confirmed by immunohistochemistry and electron microscopic studies. In situ hybridization showed large amounts of Epstein-Barr virus messenger RNA within the tumor cells. Although Epstein-Barr virus-associated smooth muscle tumors have been reported in children with AIDS and after organ transplantation, we are unaware of any case report in congenital immunodeficiency disease.

Child, Preschool↗
PubMed1999

Primary desmoplastic small cell tumor of soft tissues and bone of the hand.

Desmoplastic small cell tumor (DSCT) is a high-grade malignant neoplasm that shows polyphenotypic differentiation. Its almost exclusive involvement of serosal surfaces (particularly peritoneum) has led to the consideration of a putative "mesothelioblast" as the cell of origin. Although an extraserosal case involving the brain (presumably arising from the dura) has been reported, to date no case primary in the bone or soft tissues has been documented. The authors describe a 34-year-old man who presented with a 3-year history of pain in the right hand and a recently noted mass in the hypothenar area. Open biopsy followed by wide en bloc excision in combination with index ray resection was performed. Subsequently, the patient underwent ipsilateral axillary lymph node dissection. Extensive radiologic workup at the time of presentation and 12 months later revealed no tumor in the chest or abdomen. The patient was treated with an HD-CAV chemotherapy regimen (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide) and was free of tumor until 18 months later, at which time he developed multiple metastases in the lungs. Currently, he is alive with tumor and in poor condition. The histologic sections of the mass displayed the characteristic features of DSCT involving bone and soft tissue. Immunohistochemical stains showed positivity of the tumor cells for muscle marker (desmin), neuroendocrine markers (chromogranin, synaptophysin), and epithelial markers (keratins CAM5.2, AE1:AE3, epithelial membrane antigen). Chimeric transcripts were detected by reverse transcriptase-polymerase chain reaction, indicating the presence of EWS-WT1 gene fusion, which is characteristically associated with DSCT. Sequence analysis showed in-frame fusion of EWS exon 9 to WT1 exon 8--a variant not documented in any other case. This is a unique example of DSCT primary in bone and soft tissues, which raises interesting questions about the histogenesis of this tumor type and its relationship to other small round cell tumors. Although the "mesothelioblast" hypothesis as the origin of DSCTs is attractive, it does not account for the tumors that are located in the brain or, as in this patient, in the soft tissues and bone. In addition, this patient demonstrates a rare variant of EWS-WT1 gene fusion not described in DSCT involving serosal surfaces.

Adult↗
PubMed1999

Heterotopic mesenteric ossification ('intraabdominal myositis ossificans'): report of five cases.

Intraabdominal heterotopic ossification is a very uncommon disorder. We report five new cases, review the previous literature, and discuss the clinical and pathologic features of these lesions. The clinical features of the current cases and of those previously reported are remarkably similar. All patients were middle-aged to elderly men (range, 43-80 years; mean, 61 years) who had small bowel obstruction associated with heterotopic bone formation in the small bowel mesentery, often after one or more abdominal operations. In one case, an initial diagnosis of extraosseous osteosarcoma was considered. This unusual reactive process shares many of the clinical and pathologic features of myositis ossificans, as classically described in somatic soft tissues. We propose to designate this condition heterotopic mesenteric ossification.

Adult↗
PubMed1999

Metastatic malignant melanoma resembling malignant peripheral nerve sheath tumor: report of 16 cases.

We report 16 cases of metastatic malignant melanoma presenting clinically as lymphadenopathy or a soft-tissue mass and histologically resembling malignant peripheral nerve sheath tumor (MPNST). In two cases, the metastatic malignant melanoma was preceded by a primary cutaneous malignant melanoma; in four cases, it presented synchronously with such a tumor; and in 10 cases, there was no evidence of a previous or concomitant malignant skin lesion. Histologically, the tumors were characterized by a malignant-appearing spindle cell proliferation arranged in fascicles, often accompanied by a peritheliomatous growth pattern, alternating hypercellular and hypocellular areas, numerous mitoses, and foci of necrosis. In nine cases, there was residual lymph node tissue. In none of the cases was there evidence of an anatomic connection with a nerve, a coexistent neurofibroma, or the stigmata of neurofibromatosis. Fourteen of the cases showed strong and generally diffuse immunoreactivity for S-100 protein, and five cases showed positivity for HMB-45. Four of eight patients with follow-up information died of the disease. Tumors with a microscopic appearance compatible with MPNST but showing strong diffuse S-100 protein staining and featuring remnants of lymph node may represent metastatic malignant melanomas and should elicit a search for a previous or concomitant tumor in the skin and other sites. The similarities these tumors share with MPNST are probably related to their common neuroectodermal histogenesis.

Adolescent↗
PubMed1999

[18F]-2-fluoro-2-deoxy-D-glucose positron emission tomography localizes residual thyroid cancer in patients with negative diagnostic (131)I whole body scans and elevated serum thyroglobulin levels.

Progressive dedifferentiation of thyroid cancer cells leads to a loss of iodine-concentrating ability, with resultant false negative, whole body radioactive iodine scans in approximately 20% of all differentiated metastatic thyroid cancer lesions. We tested the hypothesis that all metastatic thyroid cancer lesions that did not concentrate iodine, but did produce thyroglobulin (Tg), could be localized by [18F]2-fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography (PET). We performed FDG-PET on 37 patients with differentiated thyroid cancer after surgery and radioiodine ablation who had negative diagnostic 131I whole body scans during routine follow-up. Serum Tg, Tg autoantibodies, neck ultrasounds, and other clinically indicated imaging procedures were performed to detect residual disease. In those with elevated Tg levels, FDG-PET localized occult disease in 71%, was false positive in one, and was false negative in five patients. The majority of false negative FDG-PET occurred in patients with minimal cervical adenopathy. Surgical resections, biopsies, 131 therapy, and differentiation therapy were performed based on the PET results. The FDG-PET result changed the clinical management in 19 of the 37 patients. In patients with elevated Tg levels, FDG-PET had a positive predictive value of 92%. In patients with low Tg levels, FDG-PET had a negative predictive value of 93%. No FDG-PET scans were positive in stage I patients; however, they were always positive in stage IV patients with elevated Tg levels. An elevated TSH level (i.e. hypothyroidism) did not increase the ability to detect lesions. FDG-PET is able to localize residual thyroid cancer lesions in patients who have negative diagnostic 131I whole body scans and elevated Tg levels, although it was not sensitive enough to detect minimal residual disease in cervical nodes.

Adenocarcinoma, Follicular↗
PubMed1999

Analysis of 35 cases of localized and diffuse tenosynovial giant cell tumor: a report from the Chromosomes and Morphology (CHAMP) study group.

The karyotypes of 44 specimens from 35 patients with localized (n = 19) or diffuse (n = 16) tenosynovial giant cell tumors were studied. The majority of cases in both categories (11 of 19 localized; 12 of 16 diffuse) displayed clonal chromosomal aberrations, with a complex karyotype in three cases and a simple chromosomal aberration in the others. No difference in the distribution of karyotypic abnormalities was found between the localized and diffuse form except for trisomies (usually of chromosomes 5 and/or 7), which were more frequent in the diffuse type. The short arm of chromosome 1 (1p11-13) was most frequently rearranged, with 7 of 11 localized and 7 of 12 diffuse lesions affected. These findings indicate that the localized and diffuse forms of tenosynovial giant cell tumor might represent two morphologic manifestations of the same entity. The high frequency of clonal chromosomal abnormalities, with a clustering of structural rearrangements to 1p11-13, suggests that this disease is most likely neoplastic in nature and paves the way to search for gene(s) that might be involved in its development.

Adolescent↗
PubMed1999

Molecular detection of micrometastases and circulating tumor cells in solid tumors.

The detection of circulating tumor cells and micrometastases may have important prognostic and therapeutic implications. Because their numbers can be very small, these tumor cells are not easily detected using conventional methods. In the last decade, molecular techniques have been widely used for the detection of occult tumor cells. The objective of this report is the application of these molecular tools to solid tumors. A systematic review of all related English-language articles published in the last 32 years was performed. The molecular detection of occult tumor cells can be accomplished by PCR amplification of tumor-specific abnormalities present in the DNA or mRNA of malignant cells. The other main PCR strategy for the detection of CTC and micrometastases involves amplification of tissue-specific mRNA. This latter method was often applied to solid tumors, whereas the former was occasionally used. PCR was shown to be superior to conventional techniques in detecting occult tumor cells, allowing the identification of 1 malignant cell mixed with 1 to 10 million normal cells. In some reports, PCR is shown to be a strong predictor of outcome. The molecular detection of circulating tumor cells and micrometastases in solid tumors can be accomplished using highly sensitive PCR assays. The central question of whether PCR reliably predicts relapse and survival remains unanswered for many types of solid tumor. If PCR-based assays are found to be a reliable tool, they will likely have a major impact on the management of these malignancies.

False Negative Reactions↗
PubMed1998

Comparison of chromosomal patterns with clinical features in 165 lipomas: a report of the CHAMP study group.

Soft tissue lipomatous tumors are morphologically heterogeneous. Various morphologic features are associated with specific chromosomal patterns and clinical features such as age, sex, and tumor site, location, and size. Simple lipomas are known to be karyotypically heterogeneous, but this has not been correlated with clinicopathological features. In 165 cases of solitary soft tissue lipoma, short-term cultures were analyzed cytogenetically. The karyotypes were divided into the following groups: normal karyotype; 12q13-15 aberrations; 6p rearrangements; 13q rearrangements, 8q11-13 aberrations; ring or giant marker chromosomes or both; other aberrations. The tumors were reexamined morphologically without knowledge of the karyotypic or clinical data. An abnormal chromosomal pattern was observed in 129 of 165 cases (78%): in 75 of 90 (83%) lipomas in the extremities and in 43 of 63 (68%) trunk wall lipomas. Chromosomal aberrations were present in 69 of 90 (77%) subcutaneous tumors and in 59 of 64 (80%) deep tumors. A normal karyotype was twice as frequent in tumors in patients under 30 years of age than in those from older individuals (6 of 16 vs. 30 of 149, 40% resp. 20%). Apart from the finding that normal karyotypes were more common in patients younger than 30 years, there was no significant association between cytogenetic pattern and patient sex or age or tumor localization, size, or depth. The pathogenetic basis and clinicopathologic relevance (if any) of the cytogenetic subtypes among benign lipomas remain unexplained.

Adolescent↗