Category Archives: Bone

Gannon FH, Glaser D, Caron R, Thompson LD, Shore EM, Kaplan FS.

Hum Pathol. 2001 Aug;32(8):842-8.

Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification associated with dysregulated production of bone morphogenetic protein 4 (BMP4), a potent osteogenic morphogen. Postnatal heterotopic ossification in FOP is often heralded by hectic episodes of severe post-traumatic connective tissue swelling and intramuscular edema, followed by an intense and highly angiogenic fibroproliferative mass. The abrupt appearance, intense size, and rapid intrafascial spread of the edematous preosseous fibroproliferative lesions implicate a dysregulated wound response mechanism and suggest that cells and mediators involved in inflammation and tissue repair may be conscripted in the growth and progression of FOP lesions. The central and coordinate role of inflammatory mast cells and their mediators in tissue edema, wound repair, fibrogenesis, angiogenesis, and tumor invasion prompted us to investigate the potential involvement of mast cells in the pathology of FOP lesions. We show that inflammatory mast cells are present at every stage of the development of FOP lesions and are most pronounced at the highly vascular fibroproliferative stage. Mast cell density at the periphery of FOP lesional tissue is 40- to 150-fold greater than in normal control skeletal muscle or in uninvolved skeletal muscle from FOP patients and 10- to 40-fold greater than in any other inflammatory myopathy examined. These findings document mobilization and activation of inflammatory mast cells in the pathology of FOP lesions and provide a novel and previously unrecognized target for pharmacologic intervention in this extremely disabling disease.

PubMed ID: 11521229

Article Size: 2 MB

 

Gannon FH, Thompson LD.

Ear Nose Throat J. 2005 Nov;84(11):694.

FIRST PARAGRAPH: The proper treatment and clinical management of osteomyelitis (bone infection) depend on a successful correlation of its clinical features with radiologic and pathologic findings. Diagnostic difficulties may arise, and the final arbiter is intraoperative culture. The importance of intraoperative culture obtained in a ‘sterile’ environment cannot be overemphasized.

PubMed ID: 16381128

Article Size: <1 MB

Nelson BL, Thompson LD.

Head Neck Pathol. 2014 Jun;8(2):168-70.

A case of an amelobastic fibro-odontoma affecting a 15 year-old girl will be discussed. The characteristic radiologic and histologic features of the entity will be described.

PubMed ID: 24197722

Article Size: <1 MB

 

Gannon FH, Thompson L.

Ear Nose Throat J. 2007 Apr;86(4):200

FIRST PARAGRAPH: Bones of the craniofacial region are frequently broken, traumatically or iatrogenically. Whereas traumatic fractures can be readily identified clinically and radiologically, they can represent a diagnostic challenge histologically. A short discussion about the histologic evolution of traumatic fractures will help a pathologist know what to expect histologically, based on the time frames of development. The repair process follows a predictable histologic evolution of five distinct phases: culatory, cellular, vascular, metabolic, and mechanical.

PubMed ID: 17500387

Article Size: <1 MB

Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD.

Cancer. 2000 May 1;88(9):2181-8.

BACKGROUND: Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors’ knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors.

METHODS: Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained.

RESULTS: The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determined by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n=1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/ recurrent tumor (at 16.6 years).

CONCLUSIONS: Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations.

PubMed ID: 10813732

Article Size: <1 MB

 

Gadwal SR, Gannon FH, Fanburg-Smith JC, Becoskie EM, Thompson LD.

Cancer. 2001 Feb 1;91(3):598-605.

BACKGROUND: Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are rare. The literature contains several single cases and small study series; however, to the authors’s knowledge, there has been no comprehensive large study to evaluate the clinicopathologic aspects of these tumors.

METHODS: Twenty-two cases of osteosarcomas of the head and neck in patients 18 years of age or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology (AFIP). No secondary sarcomas (radiation-induced or those arising after chemotherapy) or those associated with known syndromes were included. Clinical, radiographic, and histologic features were reviewed, and patient follow-up was obtained.

RESULTS: The patients included 11 girls and 11 boys, 1-18 years of age (mean, 12.2 yrs). Patient symptoms related to tumor location were painless swelling, loss of teeth, headaches, or a mass lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequently involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) and the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4.5 cm). All tumors were invasive and malignant by radiology and/or histology. The tumors were Grade 1 (n = 11), Grade 2 (n = 8), or Grade 3 (n = 3). All cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarcomas. Thirteen patients underwent initial surgical resection with (n = 5) or without (n = 9) additional radiation and/or chemotherapy. The remaining 9 patients had an initial biopsy for diagnosis followed by surgery (n = 4) or surgery and radiation and/or chemotherapy (n = 5). Follow-up was available for 19 patients: 13 were alive at last follow-up with no evidence of disease (mean, 13.1 yrs); 1 was alive with disease (1.3 yrs); 3 had died without evidence of disease (mean, 23.2 yrs); and 2 had died of disease (mean, 7.8 yrs). The 3 patients with high-grade osteosarcoma were alive without disease (mean, 20.0 yrs).

CONCLUSIONS: Primary head and neck osteosarcomas in the pediatric population are typically low- to moderate-grade lesions in the mandible. Despite the invasive nature and high grade of a few of these tumors, there is an excellent overall long-term prognosis for patients in this age group with tumors in these locations.

PubMed ID: 11169944

Article Size: <1 MB