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Pigmented villonodular synovitis is a benign disease of the synovial membrane of joints, tendon sheaths, or bursae, which nevertheless can cause marked local destruction. Its diagnosis is often delayed because complaints and symptoms are nonspecific. Familiarity with the disease may ensure an earlier diagnosis and consequently early onset of therapy, which may prevent serious damage. This paper describes 18 patients suffering from localized or diffuse pigmented villonodular synovitis. Findings possibly suggestive of pigmented villonodular synovitis include hemarthrosis, soft tissue swelling, radiological evidence of cyst formation at a distance from the weight-bearing area of a joint, an increased triglyceride concentration, and a positive bone scan. A normal appearance on arthroscopy does not rule out the disease. Therapeutic results are better in the localized than in the diffuse form of the disease.

Although generally benign, meningiomas may be associated with extensive peritumoral brain edema, as visualized on computed tomographic (CT) scans. An analysis of 38 meningiomas indicated that severity of edema on CT scans positively correlated with tumor size and also with evidence of disruption of the cortical layer, which initially separates the tumor from the white matter, in which edema tends to accumulate. The various histological subtypes also seemed to be distinct in their tendency to induce edema, with the transitional and meningotheliomatous subtypes associated with the more severe grades of edema. There was no correlation between grade of edema and location of the tumor. Contact of the edematous area or the tumor itself with the ventricle, which is relevant to the possibility of drainage of edema fluid into the ventricle, seemed to occur in cases of the more severe grades of edema rather than in cases showing slight or no edema.

A group of 424 lymphomas diagnosed as centroblastic-centrocytic lymphomas at the Lymph Node Registry in Kiel was subdivided into small (S), mixed (M) and large (L) cell groups, according to the "working formulation" proposed in a National Cancer Institute sponsored study. Histological epidemiological and clinical parameters were studied. It was found that in group S a follicular growth pattern was most frequent and in group L a follicular and diffuse growth, while group M took an intermediate position. No statistically significant differences were found in respect to epidemiological factors or overall survival. However, in the first 6 years after the diagnosis the survival in group S was better than in group M, but thereafter a reversal occurred. Group L appeared to have the worst survival throughout. Growth pattern and sclerosis were found to be of limited influence on survival within the cytological groups.

Inleiding: Steeds meer faculteiten en instellingen formuleren onderwijskwalificaties voor docenten en bieden in dit kader didactische trainingen aan. Bij volwassen leren is het belangrijk dat de deelnemers gemotiveerd zijn en dat het geleerde meteen gebruikt kan worden. Om te achterhalen wat de mening en behoefte van de docent is betreffende didactische bijen nascholing is een enquete gehouden onder docenten.

Door de Groningse Faculteit der Medische Wetenschappen is een feedbacksysteem voor toetsvragenmakers ontwikkeld. Het systeem is onder andere gebaseerd op een statistische analyse van de toetsresultaten. Uit een eerste peiling onder toetsvragenmakers blijken de respondenten overwegend positief te zijn over het ontvangen van feedback. Het ontwikkelde feedbacksysteem is echter nog voor verbetering vatbaar. Om het te kunnen optimaliseren, worden de suggesties van de respondenten als handvat gebruikt. Het specificeren van de feedbackinformatie en het verhelderen van de procedures rond de eliminatie van toetsvragen zijn daarbij twee van de belangrijkste aandachtspunten. (Reinders JJ, Cohen-Schotanus J, Molenaar WM. De ontwikkeling van een feedbacksysteem voor toetsvragenmakers. Tijdschrift voor Medisch Onderwijs 2005;24(1):14-23.)

A patient is described with Riedel's thyroiditis and invasive fibrous growth in parathyroid, lacrimal glands, and retroperitoneally. It is proposed that Riedel's thyroiditis is not a disease in its own right but a manifestation of a generalized disease of fibrous tissues.

An analysis was made of the rhabdomyosarcomas diagnosed in the Dept. of Pathology of the University of Groningen between 1971 and 1983. Ten cases diagnosed in patients over 30 years of age were studied in detail. After review the diagnosis was discarded on morphologic criteria in all cases. In 9 cases it was changed into malignant fibrous histiocytoma (MFH) and in one case this diagnosis was favoured, but inconclusive. In 5 cases immunohistochemical studies could be performed. In all cases staining for the muscle specific intermediate filaments desmin appeared negative and for the mesenchymal intermediate filaments vimentin positive. These cases were also positive for one or more of the histiocytic markers alpha-1-antichymotrypsin, alpha-1-antitrypsin and lysozyme. It is concluded that rhabdomyosarcoma in older patients is extremely rare and the possible relationship between MFH in the adult and rhabdomyosarcoma in childhood is discussed.

In children with Wilms' tumours the length of survival is greatly influenced by success in preventing or controlling metastatic disease. The current study focuses on the morphological aspects of metastases when compared with the primary tumour. In 8 patients it appeared that blastema is the most likely component to metastasize, whereas epithelial and stromal components were hardly, if at all, represented in metastases. Furthermore, flow cytometric DNA ploidy determinations on 4 cases showed that both the primary tumours and the metastases had stemlines in the diploid and low aneuploid (hyperdiploid) range. Finally, in four cases the influence of therapy on morphology of the primary tumours was analyzed. In these cases blastema seemed to be the component most sensitive to therapy. Thus, blastema seems to play a central role in prognosis of Wilms' tumours; either reacting to therapy or, if insensitive, by metastasizing.

A patient is presented with a large dermal cylindroma of the whole scalp. The origin of the tumor from sweat glands has been well accepted, but it is still not clear whether the tumor arises from the eccrine or the apocrine glands. Differential diagnosis and surgical treatment are discussed together with the typical clinical and pathological features of this tumor.

The localization and size of a high-grade soft tissue sarcoma of an extremity are generally the limiting factor in limb-saving surgery. Since 1982 nine patients with a high-grade soft tissue sarcoma of an extremity, which usually requires amputation, have been treated by intraarterial chemotherapy, preoperative and postoperative radiotherapy, and surgery. The limb was saved in eight patients (89%). During a median follow-up of 24 months (mean follow-up 32 months, range 12 to 64 months) one local recurrence and four distant metastases were diagnosed. Three patients developed complications due to the intraarterial chemotherapy, a motor and sensory neuropathy of the sciatic nerve was diagnosed in one patient, and two patients developed a flexion contracture of the knee. The results obtained in this small series show that the combination of intraarterial doxorubicin, preoperative and postoperative radiotherapy, and surgery is feasible in limb-saving treatment of primarily "unresectable" high-grade soft tissue sarcomas of the extremities without increasing the risk of a local recurrence.

A new method of identifying and locating lymph nodes in lymph node dissection specimens using a radiographic imaging technique is described. The specimen is immersed in 96% ethyl alcohol which possesses a radiographic density similar to fat tissue. Even small immersed lymph nodes contrast well with 96% ethyl alcohol. Thus, a radiograph of a nodal dissection specimen, immersed in 96% ethyl alcohol, does not show the interfering projection of variations in thickness of the specimen. Simultaneously, lymph nodes are clearly imaged. Using this procedure, we could visualize 97% of all lymph nodes present in the fat tissue of lymph node dissection specimens, which were dissected free from large radiodense structures like muscle and submandibular glands. Thus, a simple method was established for radiographic documentation of lymph nodes in lymph node dissection specimens.

Histologic material from 44 patients with follicular center cell lymphomas with a follicular growth pattern was divided into five groups on the basis of the predominating neoplastic cell type(s), i.e., small centrocytes with occasional centroblasts (SCC), centrocytes and few (CBCC/A) or many (CBCC/B) centroblasts, small and large centrocytes (SLCC), and small and large centroblasts (SLCB). Histologic, immunologic, and enzymehistochemical parameters as observed in these groups were compared, and follow-up material and material obtained during staging procedures were studied. Immunologic and enzymehistochemical findings confirmed both the B-cell origin and the neoplastic nature of the lymphomas, but did not yield relevant differences between the various groups. The groups with a predominance of small centrocytes or of small centrocytes and centroblasts showed the most prominent follicular growth and early dissemination to bone marrow and spleen. Histologic transformation in these groups was characterized by an increase in the number of centroblasts and a more diffuse growth pattern. The groups composed of small and large centrocytes or centroblasts tended to a more diffuse growth and had later dissemination and no histological transformation.

Seventy-one tumors of the central nervous system in children were studied immunohistologically. Thirty-seven were classified histologically as PNETs, of which 35 were located in the cerebellum (medulloblastomas), one in the cerebrum, and one in the spinal cord. The 34 non-PNETs included five ependymomas, seven gangliogliomas, 15 astrocytomas, and seven tumors of other histology. We used monoclonal antibodies specific for neurofilament (NF) triplet proteins, for microtubule associated protein 2 and tau protein and for glial fibrillary acidic protein (GFAP) and myelin basic protein. In addition, a monoclonal antibody to epithelial membrane antigen was applied. The presence or absence of these antigens defined four major groups of PNETs: 1) PNETs not otherwise specified (10 cases), 2) PNETs with neuronal differentiation (eight cases), 3) PNETs with astrocytic differentiation (six cases), and 4) PNETs with both neuronal and astrocytic differentiation (12 cases). One case showed ependymal differentiation. The pattern of expression of NF isoforms in PNETs was reminiscent of that seen during normal mammalian development, such that phosphorylated NF-H was only present in combination with NF-M and NF-L. Among the other central nervous system tumors, all astrocytomas and gangliogliomas were positive for GFAP, and the gangliogliomas also expressed all NF isoforms. Three atypical teratoid tumors and two rhabdoid tumors showed strong positivity for epithelial membrane antigen and also for GFAP. We conclude that the differentiation antigens described here serve to distinguish PNETs from other pediatric central nervous system tumors and to identify subsets of PNETs. Accordingly, PNETs represent a heterogeneous group of pediatric brain tumors capable of neuronal and glial differentiation.

In this report, the pathologic findings and the results of cellular DNA measurements of a tumor that on first presentation seemed to be a classical parosteal osteosarcoma are described. After resection 8 months later, part of the tumor appeared to display highly malignant features. DNA flow cytometry of this part of the tumor showed an aneuploid cell population. The aggressive nature of the tumor was confirmed by the development of lung metastases approximately 1 year after resection of the primary tumor.

Cytological, histological and DNA-ploidy findings of 2 epithelioid malignant schwannomas arising in a patient with von Recklinghausen's neurofibromatosis are described. In both primary tumors, i.e. in the thigh and in the thoracic wall, origin from a neurofibromatous nerve could be established. Within 2 years after the manifestation of the first malignant tumor the patient died of widely metastasized disease. DNA-flow cytometry of several neurofibromas revealed diploid stemlines, but both malignant primary tumors and a lung metastasis appeared to be aneuploid. Presumably, the primary tumors also contained cell populations with a diploid stem line.

High-grade soft tissue sarcomas of the extremities continue to pose problems of local disease control and deaths from distant metastases. Between 1969 and 1976, eight patients with primary and six with recurrent high-grade soft tissue sarcomas of the extremities were treated by isolated regional perfusion with cytostatics and local excision. None received systemic adjuvant chemotherapy or external-beam radiotherapy. During the follow-up (median, 13 years) five patients (36%) developed distant metastases. One was cured after resection of a pulmonary metastasis. In one other patient (7%) recurrent local disease was diagnosed after 48 months; he was cured after resection of the local lesion followed by postoperative external beam radiotherapy. The actuarial 5-year and 10-year survival was 69%. Treatment caused no cardiovascular complications and there was no postoperative mortality.

The current report describes a secondary malignancy developing in a retroperitoneal mature residual lesion 6 years after chemotherapeutic treatment of a disseminated nonseminomatous testicular tumor. The histologically malignant component was not present in the primary tumor and consisted of polygonal and fusiform cells with focal tubular formations, resembling primitive neuroectodermal tissue. Immunoperoxidase staining for alpha-fetoprotein and the beta-subunit of human chorionic gonadotropin remained negative, whereas focal positivity for S100 protein was observed. Neuron specific enolase positivity was equivocal. The DNA contents of both the mature components in the primary and the metastatic retroperitoneal tumor and in the various malignant components of the primary tumor, were in the hypotriploid range. In the malignant component of the retroperitoneal metastasis, a hypertriploid peak was observed. These findings suggest further clonal evolution in a phenotypically mature, genotypically abnormal residual metastatic tumor after chemotherapy. It is stressed that the mature appearance of the residual lesions may be deceiving and that these lesions are highly susceptible to resume malignant behavior.

Seventeen soft tissue sarcomas were karyotyped and the chromosomal findings were compared with those in the literature. Some cases were easily classified (immuno)histologically, whereas others defied classification. In four cases with unequivocal histology, a "characteristic" chromosomal abnormality was found. In one case a diagnosis that was doubtful on histologic grounds was supported by the chromosomal findings, whereas in three other cases, the chromosomal pattern led to reconsideration of the histologic diagnosis. The remaining cases either showed chromosomal abnormalities with hitherto undescribed breakpoints or breakpoints described in tumors with different histology. The results extend the knowledge of chromosomal abnormalities in soft tissue sarcomas. Most importantly, the present approach demonstrates that close collaboration between the surgical pathologist and the cytogeneticist may improve the classification of soft tissue sarcomas.

The Kiel classification for the histology of malignant lymphomas distinguishes the group of follicular lymphomas as low-grade malignancies. Clinically, this proves to be a heterogeneous group. With the aim of establishing parameters giving indications for the prognosis of the patient, a retrospective analysis was made of 30 patients with a histologic diagnosis of follicular follicle center cell tumor (FCC). Neither the immunologic markers of the cells nor the stage of dissemination have prognostic significance for survival. On the basis of the predominating neoplastic cell type, a cytologic subdivision of the lymphomas was made, characterizing the following subgroups: SCC (small centrocytes with occasional centroblasts), CBCC/A (centrocytes with several centroblasts), and CBCC/B (centrocytes with many centroblasts), SLCC (small and large centrocytes), SLCB (small and large centroblasts). The actuarial survival of the total group of 30 patients was 66% after 5 years, but the survival in the cytologic subgroups ranged from 100% in group SCC to 0% in group SLCB. Comparing the groups SCC and CBCC/A with CBCC/B and SLCB gave a significantly worse prognosis for the groups with an increased number of centroblasts (i.e., CBCC/B and SLCB). Therefore it seems justified to treat patients with a predominance of centroblasts aggressively with the aim of reaching a complete remission.

The morphology and immunohistology in a case of malignant atrophic papulosis (Degos' disease), a rare vascular disorder of unknown etiology, are described. The vascular lesions affected middle class and small arteries and veins throughout the body and were histologically characterized by intimal proliferation in the absence of any appreciable inflammation. The lesions were categorized as early, intermediate or late. Early lesions consisted of cellular proliferation and edema of the intima with signs of immune complex deposition (IgM, C3). Thrombosis was occasionally present as a secondary phenomenon in the affected vessel segments. In intermediate lesions the edema decreased and smooth muscle proliferation became apparent. Late lesions consisted of acellular intimal sclerosis with hyalinization and narrowing or obliteration of the vascular lumen. The media of the vessels remained always intact. In comparing these features to the pathology and pathogenesis of other vascular disorders they resembled the vascular lesions in a murine model of lupus erythematodes in which also considerable intimal proliferation occurred with thrombotic occlusion, but without appreciable inflammation. The murine model is associated with sustained low levels of circulating immune complexes and it is tempting to assume the same for Degos' disease. The notion of an immune complex mediated non-inflammatory condition underlying this severe and often fatal vascular disorder of mainly young males may contribute to the eventual finding of a successful therapeutical regimen.

A high-grade malignant lymphoma that arose spontaneously in a C57BL mouse has been transplanted by intraperitoneal injection of a single cell suspension for over 20 years. In the current study it was characterized cytohistologically and immunologically and was found to be a high-grade malignant (immunoblastic) B-lymphoma. The distribution in the various organs and the peripheral blood was investigated after intraperitoneal and intravenous injection and the effect of splenectomy was studied. The findings suggest that the major initial proliferation of lymphoma cells occurs in the spleen both after intraperitoneal and intravenous injection. The second, final stage is characterized by leukemic infiltration throughout the body organs. The current observations are compared to those concerning the previously described, low-grade malignant murine BCL1-lymphoma.

A patient is described with Riedel's thyroiditis and invasive fibrous growth in parathyroid, lacrimal glands, and retroperitoneally. It is proposed that Riedel's thyroiditis is not a disease in its own right but a manifestation of a generalized disease of fibrous tissues.