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Biomedical subjects

J M Mirra

Publications and source records attributed to J M Mirra.

At least 55 records · Page 3Linked to original sources

Case report 715. Necrotic giant cell tumor of the femur.

We present a giant cell tumor of the distal end of the femur that exhibited unusual diaphyseal extension and atypical MRI features. MRI demonstrated differing zonal signal characteristics in the distal metaphyseal/epiphyseal versus the diaphyseal components of the tumor. It also depicted an irregular, proximal tumor margin with an unusual, enhancing, peripheral zone. The atypical MRI features may be related to the unusual finding at pathological examination of an almost entirely necrotic giant cell tumor. This massive necrosis may illustrate a stage in the evolution of some giant cell tumors to fibrous histiocytoma-like variants of giant cell tumor or to conventional, benign fibrous histiocytoma of bone.

Adult↗

Case report 658: Chondrosarcoma of the proximal phalanx of right fourth finger secondary to multiple hereditary exostoses (MHE).

A case of grade 1 chondrosarcoma of the phalanx of a finger complicating MHE is presented. Although the cytological features did not include anaplasia, other histological features, the presence of MHE, and recurrence of the tumor after local excision aided in making the correct diagnosis. This case emphasizes the importance of correlating the histological features with the radiological and clinical findings of all bone tumors, and especially cartilaginous lesions.

Adult↗

Case report 676: Malignant granular cell tumor (schwannoma, myoblastoma), disseminated.

A rare case of metastatic, lethal, malignant granular cell tumor is presented in a 77-year-old woman. Metastases involved numerous sites, including the skeleton. The key clinical and pathological findings and differential diagnosis in benign and malignant granular cell tumor were discussed, including a review from the English literature of 23 cases of malignant granular cell tumor. The differential diagnosis was considered in detail.

Adult↗

Case report 700: Primary intracortical osteosarcoma of femur, sclerosing variant, grade 1 to 2 anaplasia.

We report a case of intracortical OS of the mid-femur in a 43-year-old man. The patient is the oldest reported to date. Histologically, the tumor was a sclerosing variant of OS with extensive "normalization" of nuclei. This is the most common histological subtype of intracortical OS. The patient was treated by en bloc resection without preoperative or systemic chemotherapy and is without evidence of disease with 15 months' follow-up.

Adult↗

Endoprosthetic replacement for stage IIB osteosarcoma.

Over an eight-year period, 100 patients with IIB osteosarcoma have been managed. Eighty-one were treated with limb-salvage procedures, 78 of which involved reconstruction utilizing endoprostheses. Only 19 patients received amputation as the primary treatment for local control. Since 1984, all patients received preoperative and post-operative adjuvant chemotherapy. Those patients who received chemotherapy had a statistically significant improvement in five-year survival over those who did not (57% and 32%, respectively, p = 0.014). The functional results were good overall, with excellent results seen in distal femoral reconstruction. Twenty-eight of the 78 patients treated with endoprosthetic reconstruction experienced 30 complications (36%). Mechanical failure was the most common complication (10%) but was generally correctable. The local recurrence rate was 5% and the infection rate was 3%. Local complications were manageable. A statistical analysis revealed that the relative risk of patient death was 2.5 times higher than prosthesis failure for the 78 patients with endoprosthetic reconstruction. The intermediate-term results, with a median follow-up period of 43 months, remains encouraging.

Adolescent↗

Enchondroma protuberans. Report of a case and its distinction from chondrosarcoma and osteochondroma adjacent to an enchondroma.

Enchondroma protuberans has been defined as an exophytic enchondroma of a long bone. We contrast a case of enchondroma protuberans with two cases of coincident enchondroma and osteochondroma. Our cases and the previously published ones lead us to believe that enchondroma protuberans is probably an enchondroma variant and that its unusual growth pattern may be related to the oblique orientation of the proximal humeral epiphysis. Unlike osteochondroma, which may be treated surgically by removing the cartilage cap and, in some cases, the stalk, enchondroma protuberans requires intralesional curettage. Enchondroma protuberans must be distinguished radiographically from chondrosarcoma.

Adult↗

Limb salvage therapy for soft tissue sarcomas of the foot.

From 1974 to 1988, 20 patients with soft tissue sarcomas of the foot underwent attempted limb preservation at UCLA. Eighteen patients had localized tumor and two had metastases. Sixteen patients had previously untreated tumors and four patients had experienced 2-4 local recurrences before definitive management. Eleven patients had grade 3 sarcomas and nine had grade 2 sarcomas. Fourteen patients had an excisional biopsy and six had an incisional biopsy. At the time of definitive local treatment, nine patients had palpable tumor ranging from 2-10 cm (median 5 cm). Sixteen patients received sequential preoperative chemotherapy and irradiation followed by attempted conservative resection. Four patients underwent immediate conservative resection followed by postoperative irradiation. Preoperative chemotherapy was administered over 3 days intraarterially, intravenously or by a combination of routes. Fifteen patients received 60-90 mg doxorubicin and one received 120 mg doxorubicin plus 220 mg cisplatinum. Preoperative irradiation was delivered in 350 cGy fractions to total doses of 3500 cGy (one patient), 2800 cGy (8) or 1750 cGy (7). Postoperative irradiation doses were 4140-6480 delivered in 180-200 cGy fractions. Fifteen of 16 preoperatively treated patients had limb salvage surgery. Four of these 15 had positive histopathologic margins and none received postoperative irradiation. One patient required a primary amputation due to gross involvement of the os calcis. Three of four patients undergoing immediate conservative excision had positive margins and one had gross residual disease. Five patients received chemotherapy following local treatment: 3 adjuvantly and 2 for metastatic disease. Follow-up for salvage patients ranged from 6 to 99 months (median 36). Local control was achieved in 17 of 19 (90%). Two patients recurred in-field at 24 and 30 months. Fourteen of 15 preoperatively treated and salvaged patients maintained local control. Three of four managed with excision and postoperative irradiation were controlled. The actuarial local control at 3 years was 83%. One patient recurred in the inguinal lymph nodes and three patients died of metastatic disease. The actuarial survival and relapse-free survival at 3 years were 83% and 63%. Eleven patients developed acute complications and four had late complications. Of 14 patients surviving with local control, function was good or excellent in 86%. No patient has required an amputation for complications or a dysfunctional foot. Limb salvage therapy for a selected patient with a soft tissue sarcoma of the foot can reasonably be expected to result in a high probability of local control and useful function without compromising survival.

Adolescent↗

Widespread inflammatory response to osteoblastoma: the flare phenomenon.

A case of vertebral osteoblastoma caused a diffuse, reactive inflammatory infiltrate in two vertebrae, adjacent ribs, and the paraspinous soft tissues. The authors call this the flare phenomenon. On magnetic resonance images the diffuse inflammatory response caused a misleading appearance that simulated a malignant process (lymphoma or Ewing sarcoma). A computed tomographic myelogram was diagnostic.

Adult↗

Giant cell tumor of metacarpal treated by cryosurgery.

We report the case of a 44-year-old woman with a giant cell tumor of the fourth metacarpal head of her dominant left hand that was treated with cryoprobe freezing, curettage and bone grafting. Three years after operation the metacarpophalangeal joint remained normal both functionally and radiologically. The tumor has not recurred. Cryoprobe killing of giant cell tumors of the hand can offer effective direct tumor eradication, as well as maintaining normal bone architecture and preserving full function.

Adult↗

Desmoplastic fibroma of bone: radiographic analysis.

Desmoplastic fibroma (DF) of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. The radiographs from 107 previously published cases of DF and seven cases from the authors' institution were analyzed to better understand and define its radiographic characteristics. DF was most common in the mandible, pelvis, and femur. A geographic pattern of bone destruction, with a narrow zone of transition and nonsclerotic margins, was seen in 80 (96%) patients with intraosseous DF for whom radiographs were available (83 patients). Internal pseudotrabeculation was seen in 76 (91%). Although widening of the host bone due to gradual apposition of periosteal new bone was common, occurring in 74 (89%) patients, distinct periosteal new bone occurred in only two (2%) patients with DF of intraosseous origin. The cortex was breached in 23 (28%) patients. Three cases of DF arising in the periosteum were identified and were differentiated radiographically from desmoid tumors of intraosseous or soft-tissue origin.

Adult↗

Hemangiopericytoma of bone.

Primary hemangiopericytoma of bone is a rare malignant tumor. Forty-one cases from the literature were reviewed, and four additional cases are reported. The peak incidence is in fourth and fifth decades. The pelvis and lower extremities are the most common locations. The radiographic features are nonspecific, although angiography may aid in the diagnosis. Based on a limited number of evaluable cases, the prognosis seems to correlate with the histologic grade of the tumor. Late recurrence and distant metastases are common.

Adult↗

Primary osteosarcoma of toe phalanx: first documented case. Review of osteosarcoma of short tubular bones.

A case of a sclerosing variant of osteosarcoma of a toe phalanx is reported in a 28-year-old man. This represents the first reported case of osteosarcoma of any kind at this site. This is based on a review of 4,214 cases of conventional osteosarcoma. The reason for the extraordinary rarity in toe or hand phalanges is unknown although osteosarcoma is the second most common primary tumor of bone. Since the neoplasm had minimal signs of cytologic anaplasia, it was originally mistaken for and treated as an osteoid osteoma. The lesion recurred and extended into soft tissues. Reevaluation revealed the tumor to be an osteosarcoma, sclerosing variant with "normalization" of nuclei. The lesions that this tumor should be distinguished from are osteoid osteoma and osteoblastoma.

Adult↗