Principles of cleft nose correction

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.     

Primary synovial chondromatosis of the ankle. A case report

A case of primary diffuse synovial chondromatosis of the ankle with long-term follow-up is reported. Trauma is implicated as a precipitating factor. This unusual, distinctive synovial neoplasm presents readily recognizable pathological features (Fig. 2). Specific diagnostic criteria are presented. The differential diagnosis of primary synovial chondromatosis includes secondary reactive synovial chondromatosis and synovial chondrosarcoma. The usual sources of osteochondritic loose bodies, on the other hand, are traumatic joint disruption, osteoarthritis, and infectious disorders. Acute primary synovial chondromatosis is adequately treated by removal of the loose bodies and synovectomy. Postoperative prognosis is dependent on the stage of the disease at the time of surgery. In this acute case, the patient had minimum impairment, and ankle roentgenograms twenty years after the arthrotomy were normal (Fig. 3).     

Longitudinal research using computerized clinical databases: caveats and constraints

Synovial chondromatosis of the temporomandibular joint is a rare occurrence. Resorption of the floor of the skull base caused by the tumour with invasion into the middle cranial fossa has only been described a few times. We report on diagnosis, histologic examination and therapy of a patient with synovial chondromatosis in the articular tubercle of the right temporomandibular joint invading the middle and cranial fossa. After tumour exstirpation the defect in the floor of the middle cranial fossa was reconstructed using a split-skull graft via an intracranial approach.     

Reproducibility of the histologic diagnosis of pneumonia among a panel of four pathologists: analysis of a gold standard

STUDY OBJECTIVE: To establish a histologic diagnosis of pneumonia by consensus of a panel of pathologists, to test the interobserver and intraobserver variation in the histologic diagnosis of pneumonia, to compare the diagnostic accuracy of diagnosing pneumonia with and without preselected histologic criteria, and to establish more specific histologic criteria for the diagnosis of pneumonia. METHODS: The study group consisted of 39 patients who died after a mean of 14 days of mechanical ventilation. A postmortem open lung biopsy was performed on all patients. The tissue was reviewed independently by four pathologists who categorized the slides from each patient as showing or not showing pneumonia. Interobserver variation was calculated using the kappa statistic. Six months following the initial evaluation, the same slides were resubmitted to one of the pathologists for reevaluation to look for intraobserver error. Finally, the slides were reviewed and categorized by the criteria of Johanson et al into no pneumonia, mild, moderate, or severe bronchopneumonia. A comparison was made of the patients selected as demonstrating histologic pneumonia by each of the examinations. RESULTS: The reliability coefficient (kappa) measuring agreement among the four pathologists was good at 0.916. However, the prevalence of pneumonia as determined by each of the four pathologists varied; pathologist A, 15 of 39 (38%); pathologist B, 12 of 39 (31%); pathologist C, 9 of 39 (23%); and pathologist D, 7 of 39 (18%). Resubmitting the same slides to the same pathologist 6 months later resulted in reclassification of 2 of 39 patients. Using the histologic criteria of Johanson and colleagues, 14 patients were selected as having pneumonia compared with only nine patients selected by consensus of three of four pathologists. CONCLUSIONS: Recognition of histologic pneumonia varies among pathologists. The preselected criteria of Johanson and colleagues detected histologic pneumonia in eight of nine patients picked by consensus of pathologists, but six additional patients classified as "no histologic pneumonia" by the consensus of pathologists were judged to have histologic pneumonia by these criteria. The results established the necessity for standardization of histologic criteria for studies using biopsy as the gold standard for bacterial pneumonia. An atlas showing the criteria used in our selection was developed.     

Synovial chondromatosis of the distal radioulnar joint: a case report

The most definitive method for confirming the diagnosis of synovial chondromatosis is the identification of cartilaginous metaplasia within the synovium on histologic examination. In advanced stages of the disease, plain radiographs and magnetic resonance imaging (MRI) scans demonstrate calcified and cartilaginous bodies, respectively, but in earlier stages, plain radiographic findings may be normal and MRI scans may reveal only nonspecific increased fluid. Although rare in the wrist, synovial chondromatosis must be considered in the differential diagnosis in a patient presenting with pain and swelling of the distal radioulnar joint. Surgical exploration of the joint and synovectomy, alone or in combination with removal of loose bodies, is the recommended treatment.     

Synovitis in internal derangement of the temporomandibular joint: correlation between arthroscopic and histologic findings

PURPOSE: To define arthroscopic criteria for synovitis (SYN) reflecting specific histologic changes. Arthroscopic observations in the temporomandibular joint of patients with internal derangement were compared with histologic patterns in synovial biopsies obtained during arthroscopy. PATIENTS AND METHODS: Arthroscopic biopsies were obtained in 31 joints of 31 patients using an oriented semiblind technique. The histologic changes in the biopsies were compared with those in age-matched reference material obtained at autopsy. RESULTS: Microscopic examination of the reference material showed inflammation in three specimens (18%), whereas it showed inflammation in 22 patient specimens (71%). Biopsies from the patients also exhibited more pronounced inflammation. According to conventional criteria, arthroscopy showed mild SYN (increased vascularity) in 51%, moderate changes in 39%, and more pronounced changes in 10% of the joints. The presence of moderate or pronounced arthroscopic signs of SYN (capillary hyperemia and synovial hyperplasia), correlated well with the histologic diagnosis of SYN. This was in contrast to patients with mild arthroscopic SYN (increased vascularity). CONCLUSION: Increased vascularity seen during arthroscopy must be questioned as a sign of SYN. More reliable criteria seem to be capillary hyperemia and synovial hyperplasia.     

Synovial chondromatosis of the temporomandibular joint with intracranial extension

An unusual case of synovial chondromatosis of the temporomandibular joint with intracranial extension, which resulted in complete dehiscence of the floor of the middle cranial fossa, is reported. An overview of the current literature and a discussion of the diagnosis and surgical management of synovial chondromatosis are presented.     

Tumours and pseudotumorous lesions of the temporomandibular joint: a diagnostic challenge

Tumours and pseudotumorous lesions originating from the synovial membrane of the temporomandibular joint are rare. We report a series of six cases of such disorders. There were two cases of synovial chondromatosis, two of calcium pyrophosphate dihydrate crystal deposition disease, one nodular synovitis and one synovial sarcoma. Three patients were female and three were male. Their ages ranged from 36 to 70 years. All had atypical clinical and radiographical presentation. The prevalence, clinical and radiographical findings and pathological features of each disease entity are discussed and a review of the literature is made concerning all tumours and pseudotumours arising from the temporomandibular joint.     

Chondrosarcoma of the short tubular bones of the hands and feet

OBJECTIVE: To report 12 cases of chondrosarcoma in a rare location, the short tubular bones of the hands and feet, as well as 12 cases of enchondroma in similar locations, emphasizing the radiologic and histopathologic features. DESIGN AND PATIENTS: All relevant cases that had both histologic slides and radiographs available were taken from the files of one orthopedic referral hospital and the personal files of one of the authors. A similar number of enchondroma cases was selected at random from the files. RESULTS AND CONCLUSIONS: One malignancy arose in a background of enchondromatosis, with all the others being solitary lesions. A pathologic diagnosis of malignancy is often difficult in the absence of radiologic signs of malignancy (cortical destruction with or without soft tissue extension). However, three cases were unusual in that the initial radiograph demonstrated a benign appearance. Another group of three malignancies was surprisingly indolent biologically. The treatment of choice is ray resection (or more limited amputation in a lesion of the middle or distal phalanx).     

Mesenchymal chondrosarcoma of the orbit. Report of three new cases and review of the literature

BACKGROUND: Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchymal chondrosarcoma have been reported. METHODS: The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed to determine clinical characteristics and appropriate therapy. RESULTS: Nine of the 10 patients were female; age of onset ranged from 10 to 35 years. Of eight patients with at least 2 years of observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct histologic types that might guide therapy. Presenting symptoms were typical of symptoms of an orbital mass: proptosis, pain, diplopia, change in visual acuity, ptosis, and tearing. CONCLUSION: The small number of reported cases of mesenchymal chondrosarcoma of the orbit prevents definitive conclusions, but it appears that resection is adequate therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of the orbit may have a better prognosis than tumors in other sites.     

The use of cryosurgery in the treatment of low and medium grade chondrosarcoma. A preliminary report

Eighteen chondrosarcoma (Grade I and Grade II) have been treated with cryosurgery. One patient subsequently developed a metastasis; three later had to have an en bloc resection (in two of these there was no tumor in the specimen) and three underwent amputation (one of these had tumor in the specimen). In general, cryosurgery for low grade chondrosarcoma is encouraging as long as the limb is protected postoperatively from fracture -- using various orthopedic appliances to allow bone regeneration (i.e. ischial weight bearing brace). Other complications such as nerve paralysis have been transient. One air embolis death occurred (in another disease) where the exit of nitrogen gas was blocked. This is preventable by allowing free exit of gas from the bone. Approximately 500 cases of primary bone have been treated with cryosurgery as of this date; long term follow-up results will be reported later.     

Low-grade myxoid chondrosarcoma of the temporal bone: differential diagnosis and report of two cases

Skull base chondrosarcoma and chordoma are rare tumors that generally have a poor prognosis. In 1973, Heffelfinger et al described a chondroid variant of chordoma, called chondroid chordoma that was found to have a significantly better prognosis than classic chordoma. However, recent evidence suggests that many of the tumors diagnosed as chondroid chordoma may, in fact, be low-grade myxoid chondrosarcomas. This report presents the diagnosis and treatment of two cases of skull base tumor that were diagnosed preoperatively as schwannoma because they were thought initially to be centred on the jugular foramen. Initial histologic evaluation suggested chondroid chordoma, but immunohistologic techniques and a review of the literature led to a diagnosis of low-grade myxoid chondrosarcoma.     

隐睾下降固定术后睾丸恶变临床分析

Objective: We summarized the relationship between the descent of a testicle into the scrotum and testicular cancer. Methods: Twenty-eight patients with testicular cancer after surgical treatment of cryptorchidism were retrospective analysis. Results:All patients were performed surgical treatment of cryptorchidism from 2 to 28 years old (median, 12 years;average, 16 years). Testicular cancer age ranged from 19 to 53 years (median, 33 years; average, 36 years). Malignant transformation occurred from 3 to 25 years of operation time (average, 18 years). Twenty-seven cases of malignant cryptorchidism ipsilateral, contralateral malignancy in 1 case, 27 cases were underwent radical resection of testicular cancer. Pathology diagnosis was mainly seminoma. Retroperitoneal lymph node dissection was done in 3 cases, 18 cases were chemotherapy and radiotherapy in 3 cases. Conclusion: The undescended testicle is the most common genital malformation in boys. When diagnosed, it should be treated as early as possible, but successful treatment appears not to lessen the risk of testicular cancer, patients must be closely monitored follow-up.     

Invasive squamous cell carcinoma arising from asymptomatic choristomatous cysts of the orbit. Two cases and a review of the literature

BACKGROUND: Epithelial choristomatous cysts are common orbital lesions, the most frequent of which are dermoid or epidermoid tumors. Massive enlargement or extraorbital extension of these benign lesions may occur. Malignant transformation of the epithelial lining of epidermoid cysts is rarely reported. METHODS: Two patients are presented in whom the epithelial lining of a previously asymptomatic choristomatous cyst of the orbit underwent malignant transformation to produce invasive squamous cell carcinoma. The unusual origin and clinical presentation of the lesions caused a delay in the diagnosis and therapy in both patients. Metastatic workup was negative in both patients, who underwent resection of their tumors via orbital exenteration with craniofacial resection. Reported cases of malignant transformation in analogous choristomatous cysts elsewhere in the cranium are reviewed. RESULTS: One patient is alive and well without recurrent disease 40 months postoperatively. The second patient died of a pulmonary embolus 2 months postoperatively. Autopsy showed no residual tumor. Overall, only 3 of 18 reported patients with epidermoid choristoma of the head and orbit with malignant transformation were alive when reported. CONCLUSIONS: Malignant squamous metaplasia is believed to be a rare complication of orbital dermoid or epidermoid cysts, with only two previously reported cases. However, malignant transformation is relatively frequent in analogous epidermoid cysts found elsewhere in the cranial vault, especially after incomplete excision. These reports encourage the complete removal of epithelial choristomas of the orbit.     

A case report. Chondromatosis of the temporomandibular joint

Synovial chondromatosis is a rare pathological condition that usually affects large joints but can affect the temporomandibular joint. The disease typically manifests itself with signs and symptoms similar to internal derangement. The disease is characterized by free-floating or attached cartilaginous bodies in the joint space. In this article, the authors present a case of synovial chondromatosis and discuss its pathological process. They also discuss diagnostic approaches and current treatment.     

MUJA: manipulation under joint anesthesia/analgesia: a treatment approach for recalcitrant low back pain of synovial joint origin

OBJECTIVE: These four cases show how application of manipulation under joint anesthesia/analgesia (MUJA) may benefit the patient with low back pain (LBP) of synovial joint origin when prior treatment options fail. We propose that MUJA should be considered as a treatment option for those with recalcitrant synovial joint-mediated LBP. CLINICAL FEATURES: We report four cases of patients with LBP successfully treated by this protocol. These four patients were neurologically normal and suffered from lumbar zygapophyseal joint and/or sacroiliac joint mediated pain diagnosed by fluoroscopically guided, contrast enhanced, intra-articular anesthetic injections. There were various radiographic findings. INTERVENTION AND OUTCOMES: All four individuals were treated with manipulation after facilitatory intra-articular anesthetic/corticosteroid injections into lumbar zygapophyseal and/or sacroiliac joints. Some also required physical therapy interventions to resolve their LBP. CONCLUSIONS: Anecdotal success with MUJA is enticing; MUJA appears to be a viable alternative for those patients in whom traditional treatment options for LBP of synovial joint origin have failed. However, prospective, quality research is required before MUJA can be formally validated or not in the treatment of documented lumbar zygapophyseal joint and/or sacroiliac joint mediated LBP.     

Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases

PURPOSE: Teratomas with malignant transformation comprise up to 6% of metastatic teratomas. The prognosis of patients with these tumors can vary considerably. We delineate factors that may be related to prognosis in a cohort of men with teratoma with malignant transformation. MATERIALS AND METHODS: We analyzed pathological features, treatment, response, recurrence, time to recurrence, subsequent followup and survival for 21 patients (median age 28 years) diagnosed with teratoma with malignant transformation during a 7-year period at our institution. RESULTS: Malignant nongerm cell elements were present in the primary tumor in 11 cases (52%). Of 18 patients with testicular primaries 17 (94%) presented with metastatic disease. Despite aggressive treatment with surgery and chemotherapy 17 of 21 cases (81%) recurred (median time 6 months). Overall, 5 patients (24%) died of disease (median survival 23 months), 5 (24%) are alive with metastases (median followup 41 months) and 11 (52%) have no evidence of disease (median followup 50 months). Progression/recurrence was substantially greater for 2 of 2 cases with a mediastinal origin, 3 of 4 with rhabdomyosarcomatous differentiation and 5 of 6 with neural differentiation compared with the remainder of the cohort (p < 0.05). CONCLUSIONS: Teratomas with malignant transformation are usually metastatic at presentation, have a high recurrence rate and are more aggressive than teratomas without malignant transformation. Prognosis is especially poor for mediastinal teratomas with malignant transformation and for those with neural or rhabdomyosarcomatous differentiation. Complete surgical resection of residual or recurrent disease appears to offer the best chance for prolonged survival.     

Isolated middle lobe atelectasis: aetiology, pathogenesis, and treatment of the so-called middle lobe syndrome

Isolated atelectasis of the middle lobe has been known for many years as the "middle lobe syndrome". Several clinical studies have shown that it may bae caused by malignant tumours. A 10-year study of 135 patients with isolated middle lobe atelectasis is presented. Fifty-eight patients (43%) had malignant tumours. Of 38 who had a thoracotomy, lung resection was possible in 25. In 20 patients regional or systemic dissemination of the tumour had been diagnosed before operation. Seventy-seven patients had benign diseases, of which 74 were non-specific infections. Bronchography was performed in 46 of these cases, and all had abnormal findings in the middle lobe, eight revealing definite bronchiectasis. In three cases tuberculosis was found. In 16 cases the benign diagnosis was established at thoracotomy. Only three patients out of 58 with malignant tumours lived more than five years. Atelectasis of the middle lobe is always a sign of potential malignancy especially in patients with a previously normal chest radiograph.     

Juvenile hyaline fibromatosis

BACKGROUND: Juvenile hyaline fibromatosis is a sporadic hereditary disease with autosomal recessive mode of inheritance, characterized by the presence of nodules and tumours in the skin and soft tissues and gingival hyperplasia. The majority of patients are growth retarded, suffer from joint disorders, contractures, osteolytic lesions and have a positive family history. The disease most frequently occurs in children, but may be diagnosed also in adults. Since 1873, when the disease was described by Murray, only a few cases were introduced in the literature. THE AIM AND THE BASIS OF THE STUDY: To present the clinical pattern of a patient diagnosed at the age of 28 and describe also the histopathological, immunohistological and electron-microscopic findings of excisions from the lesions. METHODS: The pathologist in cooperation with clinicians from several disciplines established the diagnosis on the basis of typical histopathological picture and additional immunohistological tests not yet introduced in the literature. RESULTS: Tumorous lesions contained hyaline structureless matrix often with chondroid or even osteoid metaplasia, calcium salts. The matrix contained numerous fibroblastoid-like cells with eosinophilic cytoplasm, oval nucleus and often pericytoplasmic halo. ELMI investigation revealed dilated cisternae of rough endoplasmic reticulum and hypertrophic Golgi apparatus. Sporadically were particles with calcium salts density detected. Immunohistochemical tests revealed the expression of vimentin, alfa1-antitrypsin and alfa1-antichymotripsin by the tumorous cells. CONCLUSION: The authors presented an extremely sporadic childhood disease in an adult. The results are in accordance with the data from the literature. Immunohistochemical picture of "cementicles" has not yet been introduced in the available literature. (Fig. 12, Ref. 25.)     

Anorectal malignant melanoma: report of 33 cases

33 Cases of anorectal malignant melanoma (ARMM) diagnosed pathologically were treated in Tianjin Cancer Hospital from 1996 to 1994. Some of these patients were treated with abdominoperineal resection (Mile's operation) and local resection plus chemotherapy, biotherapy and radiotherapy, others were given chemotherapy only. 25 cases were followed up from six months up to six years. The results showed that the median survival time was 23.5 months in the group of Mile's, 17.5 months of local resection and 5.5 months in chemotherapy alone respectively. The authors considered that early diagnosis and treatment are the key to raise the survival rate. For the cases diagnosed pathologically the Mile's operation should be performed and groin dissection is necessary for cases of groin lymphatic metastasis.