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J Rosai

Publications and source records attributed to J Rosai.

At least 19 recordsLinked to original sources

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and delineation of two new entities.

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.

Adolescent

Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.

We have examined the microscopic appearance, immunohistochemical staining properties, and clinical behavior of 28 cases of acinar cell carcinoma of the pancreas. Two of the tumors occurred in children. The adult patients ranged in age from 40 to 81 years (mean, 62 years). Males greatly outnumbered females, and most of the patients were white. Presenting symptoms were nonspecific, and jaundice was infrequent. The frequently reported complications from increased serum lipase levels (i.e., arthralgias and subcutaneous fat necrosis) were present in only 16% of the patients. Grossly, the tumors were relatively circumscribed and fleshy, averaging 10.8 cm, with occasionally extensive hemorrhage and necrosis. Microscopically, the tumors were very cellular and characteristically lacked a desmoplastic stroma. Acinar, solid, trabecular, and glandular patterns of growth were identified; individual tumors were usually mixed. Nuclei were round to oval, with minimal pleomorphism and single prominent nucleoli. Mitotic activity was variable. In general the cytoplasm was moderately abundant, eosinophilic, and granular, but many of the solid tumors had cells with scanty cytoplasm. Characteristic periodic acid-Schiff-positive, diastase-resistant cytoplasmic granules were demonstrated in greater than 90% of the cases, and the butyrate esterase histochemical stain for lipase activity was positive in 73%. Immunohistochemically, there was positivity for trypsin in 100% of the cases, for lipase in 77%, for chymotrypsin in 38%, and for amylase in 31%. A minor endocrine component was recognized with antibodies against chromogranin or islet cell hormones in 42% of the tumors. Ultrastructurally, exocrine secretory features were present, with polarized cells showing microvillilined lumina, abundant rough endoplasmic reticulum, and 125-1,000-nm zymogen-like granules. In addition, many cases showed pleomorphic electron-dense granules measuring up to 3,500 nm and containing fibrillary internal structures. Follow-up information was available in 88% of the cases. Half of the patients had metastatic disease at presentation and an additional 23% subsequently developed metastases, which were usually restricted to the regional lymph nodes and liver. The mean survival for all cases was 18 months, with 1- and 3-year survivals of 57 and 26%, respectively. Patients presenting before age 60 years survived nearly twice as long as older patients did. Stage also influenced prognosis, whereas the histologic subtype of the tumors and the location within the pancreas correlated only weakly with survival.

Adult

Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis.

This report describes a unique clinicopathologic entity characterized as a malignant small cell tumor of the thoracopulmonary region in 20 children and adolescents (average age 14.5 years). There was a female predilection (75%) for this tumor which appeared to originate in the soft tissues of the chest wall or the peripheral lung. The neoplasm tended to recur locally and did not seem to disseminate as widely as some of the other small cell tumors of childhood (rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma and malignant lymphoma). However, the median survival was only 8 months. Electron microscopy of 3 cases suggested a neuroepithelial derivation, but, at the present, the histogeneis remains a subject for further investigation.

Adolescent

Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma.

The clinical and pathological findings of six cases of leiomyosarcoma arising from blood vessels of different caliber are described. The term vascular leiomyosarcoma, having both a topographic and morphologic significance, is proposed for these tumors. The histologic pattern is characterized by a proliferation of atypical smooth muscle cells with a large number of intermingled blood vessels. Mitoses were counted per 10 high power field (hpf) and tumors were divided in three groups I, 10 to 20 mitoses, group II, 20 to 35 mitoses, and group III, more than 35 mitoses per 10 hpf. The mitotic index seems to be the most important pathological feature on which a prognostic evaluation for vascular leiomyosarcoma can be based. Tumors in group I had neither local recurrences nor metastases; the one tumor in group II had one local recurrence, but the patient is free of disease 6 years after surgical treatment; the three tumors in group III developed distant metastases and constitutional symptoms. Vascular leiomyoma, bizarre leiomyoma, and hemangiopericytoma are included in the differential diagnosis of vascular leimyosarcoma. The possibility that vascular leiomyosarcoma arising from small vessels represents the malignant counterpart of vascular leiomyoma is proposed.

Adult

The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy.

Of 113 cases of sinus histiocytosis with massive lymphadenopathy, 13 patients had ophthalmic infiltrates. Eleven of the 13 had infiltrates in the orbital soft tissues, and five of these patients also had eyelid disease. One patient had infiltrates only within the eyelid, and one without disease in the orbit or eyelid had extensive infiltrates in the uveal tract. The microscopic differential diagnosis included a variety of lymphoreticular malignancies, storage diseases, histiocytosis X, rhinoscleroma, tuberculosis, and inflammatory pseudotumor of the orbit. These 13 patients with ophthalmic disease were similar clinically and pathologically to patients with sinus histiocytosis with massive lymphadenopathy who did not have ophthalmic disease.

Adolescent

The histiocytoid hemangiomas. A unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone, and heart.

The proposal is made that a number of previously described entities of skin, soft tissue, large vessels, bone, and heart actually constitute different manifestations of the same basic process, characterized by the proliferation of a highly distinctive type of cell descriptively identified as a "histiocytoid endothelial cell." The entities in question are angiolymphoid hyperplasia with eosinophilia and related cutaneous and subcutaneous disorders, atypical vascular proliferation of large vessels, hemangioendothelioma of bone, and endocardial benign angioreticuloma of the heart. The main cell that proliferates in all these conditions has the basic features of an endothelial cell, but also exhibits histochemical and ultrastructural characteristics that are more akin to those of a histiocyte. These unusual features could be the expression of a morphologic abnormality or represent an overgrowth of a specific and as yet undefined subpopulation of endothelial cells, such as Majno's "contractile endothelial cell." Whether this group of proliferative diseases is of a reactive or a neoplastic nature is not immediately apparent, although the latter seems more likely. However, it is clear that the behavior of these lesions, as a group, is quite indolent and even self-limited, in contrast to the aggressive behavior and often fatal outcome of the true angiosarcomas that they so closely resemble on microscopic grounds. The term "histiocytoid hemangioma" is suggested for this group of disorders.

Adolescent

Localization of myoglobin in normal and neoplastic human skeletal muscle cells using an immunoperoxidase method.

Using an immunoperoxidase method, myoglobin is localized in the cytoplasm of normal and neoplastic human skeletal muscle. The staining intensity is variable in individual cells. This can be explained by the variable concentration of myoglobin in the different fiber types of normal muscle or the different degree of differentiation in neoplastic muscle cells, respectively. A potential application of this method in tumor pathology is discussed in connection with myoglobin as a tissue-specific marker for skeletal muscle neoplasms.

Humans

Thymic hyperplasia and neoplasia: a review of current concepts.

Although the term thymic hyperplasia is used most commonly to indicate the occurrence of germinal centers in the thymus, cognizance must be taken of the fact that such centers may occur in apparently normal thymuses in both children and adults. A concept of thymic compartmentalization is proposed with origin of germinal centers in the perivascular space (extraparenchymal compartment) of the thymus. These germinal centers contain a high percentage of B lymphocytes in contrast to the true thymic parenchyma. Although the significance of germinal centers in the thymus parenchyma. Although the significance of germinal centers in the thymus in myasthenia gravis remains controversial, removal of nonneoplastic thymus in this condition is of proven therapeutic value. A variety of neoplasms originating in the thymus have previously been lumped together under the single term "thymoma." It is apparent, however, that thymoma, thymic carcinoid, various lymphomas, and germ cell tumors that arise in the thymus differ not only pathologically but also in their clinical behavior. Thymoma is regarded as an epithelial neoplasm and ultrastucturally is characterized by many desmosomes and tonofilaments. The lymphocytes do not behave in a malignant manner, and lymphomas of the thymus should be sharply separated from true thymoma. Poorly differentiated thymic carcinoma and histiocytic lymphoma may be distinguishable only by the electron microscopic demonstration of desmosomes and filaments in the thymic carcinoma. The evidence that Hodgkin's disease of the thymus ("granulomatous thymoma") is not a variant of thymoma appears overwhelming. Lymphoblastic lymphoma of the thymus is a distinctive neoplasm that is especially prevalent in teenage males. High levels of terminal transferase characterize the lymphoblasts and there is a striking tendency for leukemia to occur. Thymic carcinoid is usually nonfunctional, although one-third of the reported cases are associated with Cushing's syndrome. On light microscopy a ribbon pattern and punctate necroses are characteristic of thymic carcinoids. Electron microscopic demonstration of many dense core granules is invaluable in establishing this diagnosis. An important clue to the diagnosis of thymic seminoma (a neoplasm that shows the same radiosensitivity as its testicular counterpart) is the frequent presence of epithelioid and giant cell granulomas and germinal centers. Separation of the various thymic neoplasms described not only is justifiable on pathologic grounds but is often essential for appropriate patient investigation and treatment.

Adolescent

The nature of myospherulosis of the upper respiratory tract.

A case of myospherulosis of paranasal sinuses similar in appearance to those recently seen in St. Louis, Missouri, was examined to ascertain the nature of the sac-like structures seen in this disease. The morphologic features of these formations and their positivity with stains for hemoglobin, peroxidase, and lipofuscin strongly suggest that they represent collections of erythrocytes altered by a foreign substance. This interpretation was confirmed by experimental production of these structures by the action of tetracycline ointment on a pure preparation of human erythrocytes.

Child

Benign tumors of the liver. Pathologic examination of 45 cases.

The histologic and clinical features of 45 benign tumors of the liver (excluding hemangiomas) were reviewed. They included 21 cases of focal nodular hyperplasia, 11 cases of hepatic cell adenoma, 12 cases of bile-duct adenoma and a single case of mesenchymal hamartoma. By adhering to published histologic criteria, the cases of focal nodular hyperplasia and hepatic cell adenoma could be readily separated. Although both focal nodular hyperplasia and hepatic cell adenoma occurred predominantly in women, focal nodular hyperplasia occurred at a greater mean age, was usually asymptomatic, and the lesions tended to be smaller (surgical cases) than hepatic cell adenoma. The latter usually were symptomatic. It is emphasized that focal nodular hyperplasia and hepatic cell adenoma are distinct lesions and are separable on histologic grounds. Although the names focal nodular hyperplasia and hepatic cell adenoma are retained, the authors cannot assert that one lesion is a neoplasm, the other a reactive condition, and prefer, instead, to leave this question unresolved.

Adenoma, Bile Duct

Peritoneal foreign body granulomas to keratin in uterine adenocanthoma.

Four cases of well-differentiated adenocarcinoma with squamous metaplasia (so-called adenocanthoma) of the uterus were associated with granulomatous inflammation of the pelvic peritoneum. The inflammation was the result of a foreign-body response to keratin that desquamated from the surface of the tumor and was carried to the peritoneal cavity through the lumen of the Fallopian tubes. The absence of viable neoplastic cells and favorable follow-up examinations in these patients indicate that the presence of peritoneal keratin granulomas in association with endometrial adenoacanthoma should not be regarded as an indicator of metastatic spread.

Adenocarcinoma

The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy.

Ten patients with sinus histiocytosis with massive lymphadenopathy (SHML) also had cutaneous involvement. Seven of the ten were children. The skin lesions were solitary in three patients and multiple in seven. They were papular or nodular, up to 4 cm in diameter, and often had a xanthomatous appearance. Microscopically, they were constituted by a dermal infiltrate made up predominantly of histiocytes, plasma cells, and lymphocytes. Some of the histiocytes contained phagocytosed lymphocytes in their cytoplasm. The microscopic differential diagnosis includes dermatofibroma, xanthoma, Tangier disease, histiocytosis X, reticulohistiocytoma, juvenile xanthogranuloma, and leprosy.

Adolescent

Sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized, distinct, pseudolymphomatous benign entity with very characteristic microscopic features. Most patients are children or young adults with massive painless cervical adenopathy, although other node groups and extranodal sites often are involved. Sixteen patients with SHML involving the upper respiratory tract and/ or salivary gland are presented. Nine of the patients had ear, nose, and throat (ENT) manifestations at the time of presentation. In most cases the ENT involvement resulted in prominent clinical symptoms. Treatment included surgery, antibiotics, irradiation, chemotherapy, and steroids, frequently in combination, but no consistent pattern of response emerged from the study. These 16 patients were very similar clinically to patients with SHML who did not have ENT disease, indicating that extranodal involvement is not associated with more aggressive disease.

Adult

The fate of intraportally transplanted islets in diabetic rats. A morphologic and immunohistochemical study.

Streptozotocin-induced diabetes in the rat can be reversed by the transplantation of isogenic islets of Langerhans from neonatal donors. We studied the morphology of intraportally transplanted islets with the aid of the immunoperoxidase staining technique to identify insulin-, glucagon-, somatostatin-, and pancreatic polypeptide-containing cells at 24 hours, 48 hours, 1 week, 2 weeks, 4 weeks, 39 weeks, and 65 weeks after transplant. Embolized pancreatic tissue, composed of approximately 80% acini and 20% islets, is initially distributed throughout the liver mainly to terminal branches of the portal system. Endothelialization and organization occur rapidly with the smaller fragments and within the first 4 weeks for larger thrombi. Exocrine pancreatic elements largely disappear as islet cells move into the hepatic lobules from the portal spaces. At 65 weeks after transplant, all islet cell types can be identified within large complex islet structures. The results of this study establish the survival and continued function of all known rat pancreatic islet cell types long after transplantation and support the theory that islet transplantation may represent the most physiologic replacement of hormonal deficiencies in the diabetic recipient.

Animals

Follicular variant of thyroid papillary carcinoma: a clinicopathologic study of six cases.

The clinicopathologic features of six cases of a peculiar variant of differentiated carcinoma of the thyroid composed of follicles with or without solid areas and having a characteristics ground-glass appearance of the nuclei were studied and compared with those of conventional papillary and follicular carcinomas. This variant resembled papillary carcinoma in its biologic behavior and all morphologic features with the exception that papillae were not present. The term "papillary carcinoma, follicular variant" is proposed for this tumor type in order to emphasize its close biologic relationship with the conventional papillary carcinoma.

Adenocarcinoma

Pathology of germ cell tumors of the testis.

Testicular germ cell tumors form a complex group of interrelated entities. Problems of nomenclature remain, but there does appear to be a close agreement between the major classifications in use. The identification of clinically useful markers for certain germ cell testicular tumors has provided a major impetus in this field. Information gained from the study of these markers has promoted an understanding of the histogenesis of germ cell tumors, has improved standard nomenclature, and has stimulated progress in patient care. Radioimmunoassay techniques are now frequently used to measure beta-human chorionic gonadotrophin and alpha-fetoprotein levels in evaluating the clinical course of patients with nonseminomatous germ cell tumors of the testis. It is hoped that the use of these markers will permit earlier detection of tumor recurrence, prompt treatment, and an improved survival rate. The knowledge of the production of alpha-fetoprotein by the human yolk sac has further refined tumor classification. It is against this general background that we have outlined the standard pathology of germ cell tumors of the testis.

Choriocarcinoma