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Biomedical subjects

John D Reith

Publications and source records attributed to John D Reith.

13 recordsLinked to original sources
PubMed2006

Aneurysmal bone cyst.

OBJECTIVES: The purpose of this article is to discuss the natural history, treatment, and outcomes for patients with aneurysmal bone cysts (ABC). METHODS: Review of the pertinent literature. RESULTS: ABCs account for 1% to 2% of all primary bone tumors, usually present in the first 2 decades of life, and exhibit a slight female preponderance. The majority of patients are treated with curettage with local control rates ranging from approximately 70% to 90%. Almost all patients with recurrences are salvaged by one or more additional operations. A small subset of patients is treated with marginal or wide excision and almost all are locally controlled. A few patients with incompletely resectable, aggressive, and/or recurrent ABCs are treated with low-dose (26-30 Gy) radiotherapy (RT) and are locally controlled in approximately 90% of cases. CONCLUSIONS: The mainstay of treatment is surgery and most patients are cured with one or more operations. A small subset of patients with incompletely resectable, aggressive, and/or recurrent ABCs may be cured with low-dose RT.

Bone Cysts, Aneurysmal↗
PubMed2006

Cutaneous angiosarcoma.

OBJECTIVE: To discuss the treatment and outcomes for cutaneous angiosarcoma. METHODS: Review of the pertinent literature. RESULTS: Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. It usually arises in the scalp or face and is locally advanced at presentation. Patients are most often white, male, and elderly. A subset of patients presents with multifocal disease and/or positive regional nodes. Although the optimal treatment is surgery followed by wide-field radiotherapy (RT), the disease is frequently so extensive at diagnosis that it is not completely resectable. Even after optimal local-regional treatment, there is a relatively high likelihood of a local recurrence at the margins of the RT fields. The probability of hematogenous dissemination is relatively high. Limited data suggest that chemotherapy may be useful for palliation with progression-free survival rates ranging from 1 to 5 months. The 5-year local-regional control rates are approximately 40% to 50%, the 5-year distant metastasis-free survival rates range from 20% to 40%, and the 5-year survival rates range from 10% to 30%. CONCLUSION: Cutaneous angiosarcoma is a rare, aggressive malignancy that is optimally treated with resection and wide-field postoperative RT. The likelihood of local-regional failure is high, as is the risk of distant relapse. Chemotherapy may be useful for short-term palliation.

Combined Modality Therapy↗
PubMed2006

Pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVNS) is a rare proliferative disorder that affects the synovium in young and middle-aged adults. Although most believe that it is an inflammatory process, some believe that it is a benign neoplasm. The optimal treatment is surgery. The local recurrence rate after marginal excision for localized PVNS is low. In contrast, the local recurrence rate after open synovectomy for diffuse PVNS is relatively high. The intra-articular instillation of radioactive isotopes or external beam radiotherapy (approximately 35 Gy in 14-15 fractions over 3 weeks) may significantly improve the likelihood of local control and long-term function in patients with incompletely resected diffuse PVNS. The probability of complications after moderate-dose radiotherapy (RT) is low.

Brachytherapy↗
PubMed2005

Cytogenetic findings in clear cell chondrosarcoma.

Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy that most often arises in the epiphysis of long bones in the third and fourth decades of life. Cytogenetic studies of clear cell chondrosarcoma are few. In this study, the cytogenetic findings of 4 cases of clear cell chondrosarcoma are presented. Clonal chromosomal abnormalities were detected in 3 cases. A tumor specific anomaly was not identified, however, extra copies of chromosome 20 and loss or rearrangements of 9p appear to be recurrent.

Adult↗
PubMed2005

Stem-like cells in bone sarcomas: implications for tumorigenesis.

Bone sarcomas are a clinically and molecularly heterogeneous group of malignancies characterized by varying degrees of mesenchymal differentiation. Despite advances in medical and surgical management, survival rates for high-grade tumors have remained static at 50% to 70%. Tumor stem cells have been recently implicated in the pathogenesis of other heterogeneous, highly malignant tumors. We demonstrate here the existence of a small subpopulation of self-renewing bone sarcoma cells that are capable of forming suspended spherical, clonal colonies, also called "sarcospheres," in anchorage-independent, serum-starved conditions. These bone sarcoma cells as well as tissue specimens express activated STAT3 and the marker genes of pluripotent embryonic stem (ES) cells, Oct 3/4 and Nanog. Expression levels of Oct 3/4 and Nanog are greater in sarcospheres than in adherent cultures. A subset of bone sarcoma cells displays several surface markers of mesenchymal stem cells (Stro-1, CD105, and CD44) as well as attributes of mesodermal, ectodermal, and endodermal differentiation. Although previously documented in brain and breast tumors, our results support the extension of the cancer stem cell hypothesis to include tumors of mesenchymal lineage. Furthermore, they suggest the participation of ES cell homeobox proteins in non-germ cell tumorigenesis.

Bone Neoplasms↗
PubMed2004

Most osteomalacia-associated mesenchymal tumors are a single histopathologic entity: an analysis of 32 cases and a comprehensive review of the literature.

Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome of osteomalacia due to phosphate wasting. The phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMTMCT) is an extremely rare, distinctive tumor that is frequently associated with OO. Despite its association with OO, many PMTMCTs go unrecognized because they are erroneously diagnosed as other mesenchymal tumors. Expression of fibroblast growth factor-23 (FGF-23), a recently described protein putatively implicated in renal tubular phosphate loss, has been shown in a small number of mesenchymal tumors with known OO. The clinicopathological features of 32 mesenchymal tumors either with known OO (29) or with features suggestive of PMTMCT (3) were studied. Immunohistochemistry for cytokeratin, S-100, actin, desmin, CD34, and FGF-23 was performed. The patients (13 male, 19 female) ranged from 9 to 80 years in age (median 53 years). A long history of OO was common. The cases had been originally diagnosed as PMTMCT (15), hemangiopericytoma (HPC) (3), osteosarcoma (3), giant cell tumor (2), and other (9). The tumors occurred in a variety of soft tissue (21) and bone sites (11) and ranged from 1.7 to 14 cm. Twenty-four cases were classic PMTMCT with low cellularity, myxoid change, bland spindled cells, distinctive "grungy" calcified matrix, fat, HPC-like vessels, microcysts, hemorrhage, osteoclasts, and an incomplete rim of membranous ossification. Four of these benign-appearing PMTMCTs contained osteoid-like matrix. Three other PMTMCTs were hypercellular and cytologically atypical and were considered malignant. The 3 cases without known OO were histologically identical to the typical PMTMCT. Four cases did not resemble PMTMCT: 2 sinonasal HPC, 1 conventional HPC, and 1 sclerosing osteosarcoma. Three cases expressed actin; all other markers were negative. Expression of FGF-23 was seen in 17 of 21 cases by immunohistochemistry and in 2 of 2 cases by RT-PCR. Follow-up (25 cases, 6-348 months) indicated the following: 21 alive with no evidence of disease and with normal serum chemistry, 4 alive with disease (1 malignant PMTMCT with lung metastases). We conclude that most cases of mesenchymal tumor-associated OO, both in the present series and in the reported literature, are due to PMTMCT. Improved recognition of their histologic spectrum, including the presence of bone or osteoid-like matrix in otherwise typical cases and the existence of malignant forms, should allow distinction from other mesenchymal tumors. Recognition of PMTMCT is critical, as complete resection cures intractable OO. Immunohistochemistry and RT-PCR for FGF-23 confirm the role of this protein in PMTMCT-associated OO.

Adolescent↗
PubMed2003

Metastatic potential of dermatofibrosarcoma protuberans with fibrosarcomatous change.

Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon fibrohistiocytic tumor that is locally aggressive. DFSP is associated with frequent local recurrences but rarely metastasizes. It has been suggested that fibrosarcomatous change within a dermatofibrosarcoma protuberans (DFSP-FS) is associated with increased risk of local recurrence and distant metastases. Metastases remain rare, however, and are typically preceded by multiple local recurrences. We report a rare case of a DFSP-FS metastasis to the head of the pancreas without any preceding local recurrence.

Adult↗
PubMed2003

Epineurial pseudocysts of the intratemporal facial nerve.

OBJECTIVE: Facial nerve pseudocysts are infrequently encountered during temporal bone surgery or neurotologic evaluation. This report describes the senior author's experience with epineurial pseudocysts of the intratemporal facial nerve. STUDY DESIGN: Retrospective case review. SETTING: Academic, tertiary referral center. RESULTS: Epineurial pseudocysts were identified on computed tomography in the mastoid segment in seven patients during evaluation for profound sensorineural hearing loss or facial nerve symptoms. Histopathologic examination revealed fibroadipose tissue without nerve tissue in five cases that underwent excision. CONCLUSIONS: Facial nerve epineurial pseudocysts are uncommon, benign lesions of the mastoid segment. Surgical excision may be safely performed for facial motor symptoms or for cochlear implant placement.

Adolescent↗
PubMed2003

Grade 2 chondrosarcoma: stage I or stage II tumor?

We analyzed chondrosarcomas of bone to assess whether Grade 2 tumors are more appropriately grouped with Grade 1 chondrosarcomas or Grade 3 and dedifferentiated chondrosarcomas (Musculoskeletal Tumor Society Stage I or Stage II). A retrospective chart review identified 109 patients who presented with nonmetastatic chondrosarcoma. Data were gathered on each tumor's histologic grade, extent (intracompartmental versus extracompartmental), resection margin status, and site (axial versus appendicular). The cohort included 60 males and 49 females with a mean age of 50 years. Forty tumors were located in the axial skeleton and the remainder were distributed throughout the appendicular skeleton. Fifty-three tumors were Grade 1, 40 tumors were Grade 2, eight tumors were Grade 3, and eight tumors were dedifferentiated. Statistical analysis established grade as the only significant variable for this group of patients; extracompartmental tumor spread also correlated with outcome, but not independent of the grade. Margin status and site were not statistically significant with respect to outcome. Chi-square analysis also established that Grade 2 tumors are grouped more appropriately with the Grade 1 chondrosarcomas as Musculoskeletal Tumor Society Stage I neoplasms. These data should be considered when developing the surgical plan, particularly for patients with Grade 2 appendicular chondrosarcomas.

Adolescent↗
PubMed2003

Mineralization in musculoskeletal leiomyosarcoma: radiologic-pathologic correlation.

OBJECTIVE: Mineralization in leiomyosarcoma, a malignant tumor of smooth muscle, has not been widely recognized. In this article, we report our experience with four cases of primary leiomyosarcoma of soft tissue or bone in which mineralization was visible on either radiography or CT. In none of the cases was the diagnosis of leiomyosarcoma considered before biopsy. In one case of a soft-tissue leiomyosarcoma, the presence of mineralization was a factor that led to the misinterpretation of the needle biopsy specimen as soft-tissue osteosarcoma. CONCLUSION: Histologically, mineralization in leiomyosarcoma appears to be caused by either nonneoplastic ossification or dystrophic mineralization in the tumor. This feature can cause leiomyosarcomas to be confused with other neoplasms.

Adult↗
PubMed2002

Angiosarcoma after breast-conserving therapy: experience with hyperfractionated radiotherapy.

PURPOSE: To report our promising results of hyperfractionated radiotherapy (RT) in conjunction with surgery for angiosarcoma occurring after breast-conserving therapy for early-stage breast cancer. METHODS AND MATERIALS: Since 1997, 3 cases of angiosarcoma after breast-conserving therapy have been managed at the University of Florida. The histologic specimens in each case were reviewed and graded by one of us (J.D.R.). RESULTS: Explosive growth of discolored skin lesions coincident with histologic evidence of angiosarcoma characterized all 3 cases but was preceded by a fairly indolent period (almost 2 years) of atypical vascular hyperplasia in 2 patients. All 3 patients were treated initially with radical surgery for the angiosarcoma, but extensive recurrences were noted within 1 to 2 months of surgery. Because of the extremely rapid growth noted before and after surgery, hyperfractionated RT was used. Two of the patients underwent planned resection after RT, and neither specimen demonstrated any evidence of high-grade angiosarcoma. All 3 patients were alive without any recurrent disease 22, 38, and 39 months after treatment. CONCLUSIONS: Hyperfractionated irradiation appears to be effective treatment for rapidly proliferating angiosarcoma. For previously untreated angiosarcoma, we now recommend hyperfractionated RT followed by surgery to enhance disease control and remove as much reirradiated tissue as possible.

Aged↗
PubMed2002

Preoperative radiotherapy in the treatment of soft tissue sarcomas.

The goal of the current study was to analyze the oncologic results and wound complications in patients with soft tissue sarcomas treated with preoperative radiation therapy and en bloc resection. A standard protocol of preoperative radiotherapy was administered followed by en bloc resection in 209 patients. The protocol included needle or incisional biopsy, prospective staging, a consistent dose of radiotherapy, standardized prospective evaluation of surgical margins, and long-term followup. The target radiotherapy dose was 50.4 cGy. Twenty-nine patients had low-grade lesions, and 180 had high-grade lesions. The mean followup was 55 months. The significant wound complication rate was 22% (moderate 14%, major 7%, and amputation 1%). Wound complications were significantly higher in patients with lower extremity lesions when compared with patients with upper extremity or axial lesions, and in patients with lesions larger than 5 cm. The local recurrence rate was 11% in patients who did not have prior surgery, and 37% in patients who had prior surgery at an outside institution (17% overall). Local recurrence was increased significantly in patients who had prior surgery. At latest followup, 78 patients (47%) were continuously disease-free, six (4%) were alive with no evidence of disease, nine (5%) were alive with disease, 63 (38%) died of disease, and 11 (7%) died of another cause. The 2- and 5-year disease-specific survival rates were 77% and 67%, respectively. Overall survival was 56%. A significant decrease in survival was seen in patients who had a local recurrence, and in patients with high-grade lesions. Preoperative radiotherapy can be given safely with a wound complication rate not significantly higher than that reported for other forms of radiotherapy delivery. Local recurrence tends to be higher if surgical intervention is done before tertiary center referral. Distant disease continues to be the cause of the high mortality in these patients.

Adolescent↗