1/12. Phosphaturic mesenchymal tumor, mixed connective tissue variant (oncogenic osteomalacia).A case of tumor-induced phosphaturic osteomalacia in a 54 year old man is reported. The patient was admitted because of progressive muscle spasms with pain and weakness in the bilateral thighs. Laboratory data showed hypophosphatemia, decreased tubular resorption of phosphate (TRP), a low 1,25-dihydroxyvitamin D level, and a high serum alkaline phosphatase level. Radiologic examinations revealed multiple lesions of osteomalacia in the ribs, and a small mass in the lower posterior mediastinum. After removal of the tumor, clinical symptoms disappeared and hypophosphatemia, decreased TRP, and the 1,25-dihydroxyvitamin D level were corrected. Microscopical examination revealed that the tumor was composed of mature adipose tissues, osseous tissues, and primitive stromal zones including osteoclast-like giant cells, non-mineralized woven bone, and various sized blood vessels. Patho-physiologic observations suggested that the tumor secreted some humoral substances inhibiting 25-hydroxyvitamin D-1 alpha-hydroxylase activity, renal phosphate resorption, and parathyroid hormone production.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/12. Giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation.BACKGROUND: spermatic cord neoplasms are a rare tumor entity and, moreover, of benign behavior. Malignant tumors of the spermatic cord are mostly of mesenchymal origin. We present the unusual case of a giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation into a liposarcoma and a leiomyosarcoma. CASE REPORT: A 84-year-old male patient presented with a scrotal mass on the left side which was observed growing since 1 year and misdiagnosed as scrotal hernia or testicular hydrocele. Ultrasound and computed tomography demonstrated a solid tumor suggesting a spermatic cord tumor. The patient underwent hemiscrotectomy, and the histological examination of the 2,500-gram specimen revealed a malignant mesenchymoma originating from the spermatic cord with two distinct histopathological compartments of liposarcoma and leiomyosarcoma. Because an adjuvant therapy protocol is of questionable effect and because of the patient's age no further therapy was applied. The patient was closely followed and is now, 5 years after surgery, still free of disease. CONCLUSION: Even in older patients, scrotal masses should be considered malignant tumors as long as no benign diagnosis has been proven. Although malignant mesenchymomas are rare tumors with poor prognosis, in selected cases even large tumor masses, as presented, can be cured by surgery.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/12. Chest wall hamartoma. Report of two cases with secondary aneurysmal bone cysts.Chest wall hamartoma is a rare non-neoplastic benign lesion occurring in the neonatal period with particular clinical, radiological and pathological features. Histologically, it is composed of a mixture of bone trabeculae with spindle-cell stroma, chondroblast-like cells, and mature and immature hyaline cartilage. Aneurysmal bone cyst is a benign lesion that may arise secondary to several bone processes, such as giant cell tumor, chondrosarcoma, non-ossifying fibroma or osteosarcoma. We present two new cases of chest wall hamartoma with secondary aneurysmatic bone cyst formation studied with histological and immunocytochemical methods.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/12. Mixed mesenchymal differentiation in meningiomas.Five intracranial meningiomas showing mixed mesenchymal differentiation are described. Three contained cartilage, three contained bone, four contained hyaline fibrous strands or nodules which in two instances were calcified, three contained angiomatous areas, one contained pericytoma-like areas, four contained pleomorphic and sometimes multinucleate giant cells, and one contained a mucoid matrix including spheroidal cells superficially resembling chordoma. The importance of recognising such tumours is emphasised as they can be misdiagnosed as metastatic deposits, particularly in a frozen section.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/12. August 2003: 47-year-old female with a 7-year history of osteomalacia and hypophosphatemia.The August 2003 COM. A 47-year-old woman presented with a long history of muscle pain, weakness, and visual disturbances. Over the last year, she developed diplopia and left sixth nerve palsy. No other neuro-ophthalmologic abnormalities were found. Past medical and family history was unremarkable. Laboratory investigation disclosed hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase activity, and normal serum calcium levels. CT scans showed a lobulated mass arising on the meningeal surface of the cavernous sinus, measuring 3x 2 x 2 cm. The lesion was partially resected and microscopic examination revealed a highly vascularized tumor composed of primitive mesenchymal cells arranged whether in a patternless-pattern or surrounding thin-walled, branching vascular spaces and thick-walled hyalinized vessels. Other eye-catching features were microcystic areas, multinucleated osteoclastic-like giant cells, scattered islands of mature adipocytes, foci of hemorrhage, thrombosed medium-sized-to-large vessels, and hemosiderin-laden macrophages. After surgery, the patient recovered from the muscle pain and weakness, with a significant improvement of serum calcium and alkaline phosphatase levels and phosphate blood levels. Taken together, the clinical and pathologic features were consistent with a diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue variant) of the cavernous sinus in a setting of oncogenic osteomalacia. No further treatment was carried out. The patient has been followed for the last 4 years with no evidence of recurrent disease. Oncogenic osteomalacia is a rare clinical entity characterized by hypophosphatemia, phosphaturia, and a low concentration of 1,25-dihydroxyvitamin D(3) caused by a neoplasm. Pathologists should be aware of this entity, because surgical excision of the tumor is usually curative.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/12. Pediatric penile tumors of mesenchymal origin.OBJECTIVES: To describe 2 cases of mesenchymal pediatric penile tumors in a 13-year-old boy and an 11-month-old infant. Mesenchymal tumors are rare in the male external genitalia. methods: Two patients, one aged 13 years and one 11 months, presented with painless penile masses at the penoscrotal junction. The evaluation included serial clinical examinations and ultrasonography. Both lesions were surgically excised after increasing in size. Because of malignancy, the first patient underwent wide reexcision of the tumor site. RESULTS: The pathologic findings of the first case were combined dermatofibrosarcoma protuberans, giant cell fibroblastoma, and fibrosarcoma, a malignant tumor. Immunohistochemical staining showed strong and diffuse CD34 cytoplasmic positivity in the giant cell fibroblastoma and dermatofibrosarcoma protuberans components; the dedifferentiated fibrosarcoma tumor cells were negative for this antibody. The second case was myofibroma, a benign fibroblastic tumor with a prominent myofibroblastic component that is congenital and tends to occur within the first few months after birth. Neither patient had recurrence at 16 months and 3 years of follow-up. To our knowledge, this is the first reported case of combined dermatofibrosarcoma protuberans, giant cell fibroblastoma, and fibrosarcoma of the penis and the second reported case of isolated penile myofibroma. CONCLUSIONS: Penile tumors should be included in the differential diagnosis of pediatric penile masses. Surgical resection is often curative, but patients with malignant tumors should receive careful follow-up to monitor for recurrence.- - - - - - - - - - ranking = 3keywords = giant (Clic here for more details about this article) |
7/12. Giant benign mesenchymoma of the breast.The term mesenchymoma refers to a group of mixed tumors that are composed of two or more mesenchymal elements, excluding fibrous tissue, not ordinarily found together within the same tumor. Mesenchymomas occur most commonly in the renal and perirenal regions with rare occurrence in the breast. We describe what to our knowledge is the first report of a giant benign mesenchymoma of the breast. The clinical presentation, course, and treatment of a patient with this condition is discussed. Clinicians should be aware that benign mesenchymomas may involve the breast and simulate a malignant breast neoplasm.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
8/12. Malignant mesenchymoma of spermatic cord: a case report with intermediate filament typing.A malignant mesenchymoma arising in the spermatic cord of a 79-year-old patient is described. The radiologic appearance was that of a predominantly calcified mass. Histologically the tumor was composed primarily of malignant cartilage. In other areas there were spindle-shaped cells and large eosinophilic and multinucleated giant cells. Staining for intermediate filaments revealed vimentin throughout the neoplasm with some areas showing positive staining for desmin, the latter confirming the muscular origin of some of the neoplastic elements. No cytokeratins were identified, effectively ruling out the possibility of a malignant teratoma.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
9/12. Calcification in pulmonary metastases.A large variety of neoplasms can produce calcified lung metastases. Three unusual examples are presented and the relevant literature is reviewed. Each case involves a neoplasm not previously reported to produce calcified lung metastases: malignant mesenchymoma, fibrosarcoma of the breast, and medullary carcinoma of the thyroid. The sarcomas are reported in the literature to develop calcified lung metastases are osteogenic sarcoma, chondrosarcoma, synovial sarcoma, and giant cell tumour. Among carcinomas, the papillary and mucinous adenocarcinomas are the histological types most likely to develop calcified lung metastases. The metastases of a number of other tumours have calcified after antineoplastic therapy. Calcification in metastases arises through a variety of mechanisms: bone formation in tumour osteoid, calcification and ossification of tumour cartilage, dystrophic calcification and ossification of tumour cartilage, dystrophic calcification and mucoid calcification. Since calcified lung metastases can strongly resemble granulomas or hamartomas, a reasonable suspicion of malignancy is necessary when evaluating calcified pulmonary nodules.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/12. Malignant mesenchymomas of soft tissue associated with numerous osteoclast-like giant cells mimicking the so-called giant cell variant of "malignant fibrous histiocytoma".Three cases of malignant mesenchymoma with numerous osteoclast-like giant cells, arising in deep soft tissue, and which mimicked the so-called giant cell variant of "malignant fibrous histiocytoma" have been studied. All three neoplasms arose in adults; two patients were male and one was female. Two tumours arose in the thigh, and one in the right shoulder. Two patients died within 2 years of the primary excision while the third is alive and well at 2.5 years. Histologically, one case showed leiomyosarcoma plus liposarcoma, one leiomyosarcoma plus osteosarcoma, and one tumour consisted of liposarcoma plus osteosarcoma; all components were assessed morphologically as high-grade malignant. All three cases showed prominent osteoclast-like giant cells in the leiomyosarcomatous or osteosarcomatous areas, thereby closely mimicking the phenotype of so-called giant cell variant of "malignant fibrous histiocytoma". We discuss briefly differences in soft tissue sarcomas demonstrating this distinctive osteoclast-rich phenotype.- - - - - - - - - - ranking = 12keywords = giant (Clic here for more details about this article) |
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