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Biomedical subjects

R O van der Heul

Publications and source records attributed to R O van der Heul.

At least 19 recordsLinked to original sources

Chondrosarcoma of the phalanx: a locally aggressive lesion with minimal metastatic potential: a report of 35 cases and a review of the literature.

BACKGROUND: Enchondroma is the most common primary benign bone tumor of the hand, especially the phalanges, whereas chondrosarcoma is uncommon at this site. Although phalangeal chondrosarcoma may have ominous histologic features, its biologic behavior is relatively indolent. METHODS: Thirty-five cases of phalangeal lesions previously diagnosed as chondrosarcoma were studied. Histologic and tumor-biologic parameters (Ki-67 and p53 immunohistochemistry) were investigated and correlated with clinical behavior. RESULTS: All cases were characterized by unequivocal malignant histologic features (Grade 2 or higher) or Grade 1 malignant histologic features combined with the presence of cortical destruction and soft tissue extension. The median age of the patients at the time of diagnosis was 67 years (range 21-87 years), with a slight female predominance. Occurrence in the hand was more common than in the foot, with the proximal phalanx affected most often. Treatment varied from local therapy (curettage or local excision) in 16 patients to amputation or exarticulation in 19 cases. Follow-up ranged from 8-432 months for 28 patients. Ten of 15 tumors treated by local therapy recurred whereas none of 13 tumors treated by radical surgery recurred. The median survival was 20.8 years; none of 28 patients developed metastases nor died of disease. Both the type of treatment and localization in the proximal phalanx were associated independently with local recurrence. CONCLUSIONS: Phalangeal chondrosarcoma behaves as a locally aggressive lesion and, in contrast to chondrosarcomas located elsewhere, rarely metastasizes. Treatment is indicated only because of its locally destructive growth. The authors believe that given the excellent survival data, curettage with adequate follow-up should be considered as the treatment of choice if technically feasible, especially in those cases in which amputation would lead to a significant loss of hand function.

Adult↗

Aneurysmal bone cyst of the spine: 31 cases and the importance of the surgical approach.

Thirty-one cases of aneurysmal bone cyst of the spine with a minimum follow-up of 1 year as registered by the Netherlands Committee for Bone Tumors are reported. Aneurysmal bone cyst is a benign, sometimes expansive and destructive, lesion frequently occurring between the ages of 10 and 20 years. Located in the vertebral column, it undermines the structural integrity of the spine, and neurologic complications can occur. In all cases the posterior elements were involved, with extension into the vertebral body occurring in 22 cases. Extension to adjacent vertebra occurred 5 times, but the intervertebral disc appeared to be left intact. Twenty-nine of 31 cases were treated surgically. Posterior approaches provided insufficient access to lesions also located to the vertebral body and were associated with a higher recurrence rate (5 of 18 cases) than anterior approaches (1 of 11 cases). Of 10 cases in which the surgical removal was incomplete, 4 healed and 6 had a local recurrence. Selective embolization was used twice, and its application is discussed. On the basis of this material and a review of the literature, treatment by intralesional curettage combined by adjuvant therapy such as preoperative embolization is advised.

Adolescent↗

Synovial chondrosarcoma: report of a case and review of the literature.

Primary synovial chondromatosis is a relatively uncommon condition. Synovial chondrosarcoma is considered to be very rare and it is not always clear whether the sarcoma develops by malignant transformation of synovial chondromatosis or whether it arises de novo. Differentiation of the two conditions on clinical and radiographic features is not possible and on histological criteria can be difficult. We report the case of a 59-year-old man who, 4 years after a synovectomy for synovial chondromatosis, developed a synovial chondrosarcoma of the hip. The literature is reviewed.

Chondrosarcoma↗

Tumoral calcinosis.

A patient with tumoral calcinosis involving the buttock and ischial bone is presented. Bone marrow involvement and a pattern of septal enhancement on MR imaging after intravenous administration of Gd-DTPA were very suggestive of a diagnosis of a chondro(sarco) matous musculoskeletal tumor. The absence of an underlying metabolic disorder, the appreciation of fluid-calcium levels within the lesion and knowledge of the macroscopic and microscopic appearance of this disorder have led to the correct diagnosis in this case.

Bone Diseases↗

Desmoplastic fibroma of bone: a report of 18 cases.

Desmoplastic fibroma is a rare benign primary bone tumor, histologically identical to the extra-abdominal desmoid tumor of soft tissues. The incidence in major series of bone tumors is 0.1%-0.3%. In the present study 18 cases from the files of the Netherlands Committee on Bone Tumors are reported, with an emphasis on the radiographic features. Eight female and ten male subjects were affected, with a fairly even age distribution from the second to the seventh decade of life. The most frequent sites of involvement were the femur and the pelvis. Radiographically the lesions nearly always demonstrated benign features with respect to pattern of destruction, margins, and reactive bone formation; however, soft tissue extension was sometimes present. Although desmoplastic fibroma usually presents with pain or swelling, in 5 of the 18 cases the existence of the lesion was an accidental finding, and in 2 cases the patient had long-standing, vague complaints of pain. Though overall benign radiographic features may support the final diagnosis, the main and often troublesome histological differential diagnosis is of a low-grade fibrosoma. En bloc resection is the treatment of choice in view of the high incidence of recurrence after curettage.

Adolescent↗

Ectopic cartilage in subglottic stenosis: hamartoma or reaction to trauma?

In an experimental study in growing rabbits an endolaryngeal injury to the subglottis resulted in the development of a stenosis due to the formation of scar tissue containing ectopic cartilage. For comparison, biopsies taken from the subglottic stenosis in 8 children were studied histologically. In 6 cases ectopic cartilage was observed; all patients had a history of endotracheal intubation. In 3 children the diagnosis hamartoma was made. In the remaining 3 cases the formation of ectopic cartilage might have been a direct reaction to the endolaryngeal intubation. The observations suggest that the formation of ectopic cartilage in acquired subglottic stenosis is not always due to a developmental aberration such as a hamartoma.

Animals↗

Reactive mesenchymal proliferation.

One hundred and thirteen cases in the files of the Netherlands Committee on Bone Tumors were diagnosed as heterotopic soft tissue ossification. Myositis ossificans was diagnosed in 62, ossifying hematoma in 21, and pseudomalignant osseous tumor of soft tissues in 30 cases. Antecedent trauma was present in 37%, 46% and 7%, respectively. Myositis ossificans arose in the large muscle groups of the thighs and upper arms; when closely related to the shaft of a bone, periosteal reactions were more outspoken. Pseudomalignant osseous tumor of soft tissues was located in the hands, feet, and pelvis, some cases were not in muscle groups and some were almost periosteal. Ossifying hematoma was located in the upper and lower legs and usually in close relation to bone. The three entities belong to the same kind of reactive mesenchymal proliferative process. The radiologic and histologic patterns are reflections of whether the lesions are closely localized to bone shafts or in soft tissues, e.g. in muscles and therefore the term "reactive mesenchymal proliferation" is preferred to myositis ossificans, pseudomalignant osseous tumor of soft tissues and ossifying hematoma. Diagnostic problems are encountered in early phases when cellularity, mitotic activity, and infiltrative spread suggest malignancy. Recognition of these reactions in early phases is important to avoid mutilating surgery.

Adolescent↗

Ultrastructural and immunohistochemical segregation of gemistocytic subsets.

Gemistocytes are frequently encountered in cases of reactive gliosis as well as in glial tumors. Recently, miniature forms of gemistocytes (minigemistocytes) were recognized as cellular constituents of oligodendrogliomas. Antibodies specific for the intermediate filaments glial fibrillary acidic protein and vimentin are reactive with gemistocytic cells, but do not react specifically with these cells. In a study of 23 glial tumors we found the monoclonal antibody Pm43 selectively reactive with the classical gemistocytes as well as with the minigemistocytes. Nevertheless, at the ultrastructural level a striking difference in the arrangement of the glial filaments between both gemistocytic cell types was found. Immunoelectron microscopy showed that the reactivity for the newly discovered gemistocytic marker Pm43 was confined to identical intermediate filaments. Despite immunohistochemical homology, a clearly different ultrastructure divides classic gemistocytes and minigemistocytes into two subsets.

Adolescent↗

Histologic evaluation of crushed cartilage grafts in the growing nasal septum of young rabbits.

The evolution of crushed cartilage grafts in a growing nasal septum of young rabbits was histologically investigated. Crushing leads to necrosis of the greater part of the graft. The characteristic distribution of surviving cells in isolated areas at the surface of the graft reflects different mechanical properties of the centrally and peripherally located cartilage. The necrotic parts are eliminated and replaced by new cartilage formed by surviving chondrocytes or perichondrium. The result is a complex structure composed of two types of cartilage and of fibrous tissue without any distinct organization, but nevertheless capable of growth. However, it lacks strength to transform growth into adequate lengthening of the septum. Subsequently, this will lead to the development of multiple deviations. It is concluded that crushed cartilage is no suitable implant for the reconstruction of parts of the septum which are essential in supporting the outgrowth of the nose.

Animals↗

Wound healing of autologous implants in the nasal septal cartilage.

The cartilaginous nasal septum of the child is often involved in trauma or congenital deformation. In perspective of its role in postnatal growth of the midface, the wound healing of septal cartilage in reaction to various surgical procedures was studied in young rabbits. It was concluded that (1) elevation of mucoperichondrium does not induce significant histologic reactions in the septal cartilage and (2) the wound reaction of cut edges includes only a +/- 0.8-mm-deep zone and lasts no longer than 2 weeks, resulting in a stump, encapsulated by fibrous tissue. Reconnection of separated parts occurs 2 weeks after surgery, mostly by merging of the perichondrial layers. Reimplantation of resected cartilage does not lead to normalisation of septal growth. This is not caused by extensive necrosis, loss of morphological organisation or decreased growth potential of the reimplanted cartilage. The essential factor seems to be dislocation (duplication, angulation) of the implant versus the non-mobilised parts of the septum preventing the growth of the implant to contribute fully to the lengthening of the total septum.

Animals↗

Fibrous dysplasia vs adamantinoma of the tibia: differentiation based on discriminant analysis of clinical and plain film findings.

Differentiation between benign fibrous dysplasia and malignant adamantinoma of the tibia is challenging because of the impact the diagnosis has on the choice of treatment (none or extensive surgery). The histologic and pathologic similarities of the lesions and the controversial relationship between fibrous dysplasia, osteofibrous dysplasia, and adamantinoma complicate the matter. We found a large overlap of histologic features in lesions considered either fibrous dysplasia or osteofibrous dysplasia on the basis of the radiologic findings. The purpose of this study was to determine the value of the plain radiograph of the lower leg in combination with clinical findings to differentiate the benign from the malignant condition. The clinical symptoms, radiographs, and histologic slides of 46 patients with fibrous dysplasia and 22 with adamantinoma in the tibia were reviewed retrospectively. In only one of 12 patients with radiologic or histologic characteristics of osteofibrous dysplasia were both radiologic and histologic criteria for the diagnosis present. A linear discriminant analysis was performed on six clinical (age, spontaneous pain, pain after trauma, swelling only, pain and swelling, and bowing deformity) and 25 radiologic signs. Fibrous dysplasia and its variant osteofibrous dysplasia could be identified correctly in 87% (40 of 46 patients) and adamantinoma in 95% (21 of 22 patients) by using the patient's age and four radiologic signs. When results from the discriminant analysis of a randomized subgroup of patients (32) were used on the other subgroup (36 patients), fibrous dysplasia was correctly identified in 84% (21 of 25) and adamantinoma in 82% (nine of 11). Fibrous dysplasia is more prevalent than adamantinoma in a young patient, when radiographs show a ground-glass appearance and anterior bowing and when there is no multilayered periosteal reaction and moth-eaten destruction. When radiologic signs and the patient's age are combined, fibrous dysplasia and adamantinoma can be discriminated in a high percentage of patients.

Adolescent↗

X-chromosome inactivation in patients who have pigmented villonodular synovitis.

We performed a histological examination and DNA analysis on samples of tissue from a forty-seven-year-old woman who had a clinical diagnosis of pigmented villonodular synovitis. The histological examination confirmed the diagnosis. The evaluation of the samples of tissue with preferential X-chromosome inactivation analysis (a molecular biological method for the study of clonality in tumors) showed that the lesion was polyclonal in origin. We concluded, therefore, that pigmented villonodular synovitis is more likely to be a reactive process than a true neoplasm.

Blotting, Southern↗

Synovial sarcoma: a clinicopathological study of 36 cases.

Thirty-six cases of synovial sarcoma (13 biphasic and 23 monophasic) were subjected to a clinicopathologic study that included electron microscopy and immunohistochemistry. The group consisted of 21 males and 15 females ranging in age from 2 to 63 years. The majority of tumors (27 cases) were found in the hip and lower extremity. Immunohistochemical study revealed that keratin, which was detected in 92% of the biphasic and 57% of the monophasic tumors, was a more sensitive marker of epithelial differentiation than EMA or CEA. The overall 5-year survival of the patients was 64%. Male sex, older age, presence of tumor necrosis, monophasic pattern, and absence of keratin positivity had an unfavourable effect on survival but lacked statistical significance. Survival was significantly lower in patients with tumors exhibiting more than 15 mitoses per 10 HPF (P less than .02) and in those with tumors showing necrosis and a mitotic rate greater than 5 mitoses per 10 HPF (P less than .005).

Adolescent↗

Morphometrically estimated variation in nuclear size. A useful tool in grading prostatic cancer.

At present there are several grading systems for prostatic carcinoma. Most are difficult to reproduce. An objective method of grading seems to be necessary and could make comparisons between various groups of patients easier and grading more reliable. In the present study morphometrically estimated nuclear size and variation in nuclear size are matched with the survival rates of 207 patients who underwent total perineal prostatectomy for cancer. On the basis of morphometrically estimated variation in nuclear size the patients could be divided into two groups with significantly differing survival rates. In this way it was possible to split the group of patients with grade 2 carcinoma (Mostofi's grading system) into two groups of patients with significantly different survival rates. The survival rates in these two groups did not differ significantly from those in the patients with Grade 1 and Grade 3 tumors respectively. The results are discussed in the light of the recent literature on the subject. Morphometry seems to be a valuable tool in grading prostatic cancer.

Cell Nucleus↗

Histological grading of prostatic carcinoma in prostatectomy specimens. Comparison of prognostic accuracy of five grading systems.

The prognostic accuracy of 5 histological grading systems (Broders, Anderson, Mostofi, Gleason and Mostofi-Schroeder) was compared. Grading was performed on 50 prostatectomy specimens by 5 pathologists. The results were averaged so as to reduce the impact of inter-observer variation. The Cox proportional hazards model was used to estimate the relationship between average grading scores and both time-to-recurrence and time-to-death by prostatic carcinoma. Age at surgery was considered to be a possible confounding factor and adjusted accordingly. The prognostic impact of the 5 grading systems (related to both recurrence and death caused by prostatic carcinoma) was judged by the likelihood ratio (LR) test score (chi 2 distributed with 1 df); for time-to-recurrence for the Mostofi-Schroeder score the LR was 6.54 and for the Gleason system it was 1.79. A stepwise procedure demonstrated that the best prognostic performance was reached with the Mostofi-Schroeder and Broders systems used together (with Mostofi-Schroeder weighted 1.5 times larger than Broders). For time-to-recurrence the median grading result was also used, giving results similar to the mean grading result. For time-to-death from prostatic carcinoma the LR test scores for all grading systems were relatively low. In this analysis the outcome of the Gleason system showed a minimum of prognostic ability, whereas the Broders and Mostofi-Schroeder systems had a reasonable predictive ability. Since the inter-observer variation of the Mostofi-Schroeder system was large, the Broders system is preferable. The restrictions and implications of this study are discussed and a brief review of the prognostic importance of grading of prostatic carcinoma is presented.

Aged↗

Pseudomalignant, nonneoplastic osseous soft-tissue tumors of the hand and foot.

Seventeen patients with nonneoplastic osseous tumors in the soft tissues of the hands and feet were retrospectively studied. Three of the patients had a reliable history of trauma. Ten patients showed periosteal reaction associated with gradual formation of an osseous soft-tissue tumor, sometimes with a peripheral zone of bone density. Histologically (also radiographically), the lesions may be mistaken for malignant neoplasms such as osteosarcoma or parosteal osteosarcoma, especially when the pathologist does not receive representative material. In this series, malignancy was suspected in eight patients. Radiologic and pathologic criteria that may be helpful in the differential diagnosis of this lesion are presented. It is important that this nonneoplastic, benign, and self-limiting process in the hands and feet be recognized and treated accordingly. Local excision is adequate therapy in most cases, with little risk of local recurrence.

Adolescent↗