Claudia E. Noffke

LOW-GRADE INTRAOSSEOUS OSTEOSARCOMA OF THE JAWS

Two cases of low-grade intraosseous osteosarcomas are reported, bringing the total number of such osteosarcomas in the jawbones documented in recent literature to six. Our first case involved the maxilla of a 69-year-old man and the second involved the mandible of an 18-year-old girl. In clinical and microscopic appearance, these neoplasms resemble benign proliferations in many respects. Subtle differences include cortical bone destruction, soft tissue infiltration, irregular bone production with foci of abundant osteoid, and mild cellular atypia. Complete removal at the first attempt is of paramount importance, inasmuch as a significant percentage of these neoplasms recur as high-grade osteosarcomas if they are inadequately treated.

Internal morphology of ameloblastomas: a study of 24 resected specimens

OBJECTIVES The objectives of this study were to describe the internal macroscopic architecture of resected specimens of ameloblastoma and to correlate the findings with radiographs and microscopic features. STUDY DESIGN Resection specimens of 24 ameloblastomas were retrieved from the files of the Department of Oral Pathology at the University of Limpopo. The neoplasms were sectioned in parallel slices and the macroscopic features recorded and each slice was radiographed and sampled for microscopic examination. The macroscopic features were correlated with respective microscopic and radiological appearances. RESULTS Twenty-three ameloblastomas affected the mandible and 1 the maxilla and measured between 3.3 and 20 cm in greatest diameter. Six cases were unicystic, 2 of which showed incomplete septae both of which presented multilocular on radiographs. Intracystic proliferations were present in 15 cases. These proliferations showed macroscopic features of either small or large nodules with or without the formation of confluent plaques, focal papillary lesions, or multinodular masses that protruded into the cystic cavities. Microscopically these proliferations were characterized by foci of inflammation or plexiform or solid epithelial proliferations, one of which showed a focus of carcinoma in situ, adenomatoid differentiation and another osteodentin deposits. Seven cases had foci of stromal desmoplastic change, one of which exhibited mineralized deposits resembling bone. CONCLUSIONS The assessment of the cystic nature of ameloblastomas on 2-dimensional radiographs is inaccurate. Intraluminal proliferations, in situ carcinomatous change, adenoid differentiation, stromal osteodentin, and bone deposits and desmoplasia were found to be focal rather than generalized phenomena in resection specimen of ameloblastoma.

Peripheral Odontogenic Myxoma: A Review of the Literature and Report of Two Cases

Two cases of peripheral odontogenic myxoma with a verifiable location in gingival soft tissue and without bone involvement were compared with those reported in the literature. This study showed that they form a distinct albeit rare clinical entity with a potential to grow into large disfiguring lesions. The probability that small peripheral odontogenic myxomas are interpreted as edematous irritation fibromas may contribute to the small number of peripheral odontogenic myxomas recorded in the literature. The differential diagnosis of soft tissue myxoid proliferations is discussed.

Report of a Black South African Child With Oculodentodigital Dysplasia and a Novel GJA1 Gene Mutation

Liviu Feller,* Neil H. Wood, Michelle D. Sluiter, Claudia Noffke, Erich J. Raubenheimer, Johan Lemmer, and Elizabeth J. van Rensburg Department of Periodontology and Oral Medicine, School of Dentistry, University of Limpopo (Medunsa Campus), Pretoria, South Africa Department of Genetics, University of Pretoria, Pretoria, South Africa Section of Maxillo-Facial Radiology, School of Dentistry, University of Limpopo (Medunsa Campus), Pretoria, South Africa Department of Oral Pathology, School of Dentistry, University of Limpopo (Medunsa Campus), Pretoria, South Africa

Expansive jaw lesions in chronic kidney disease: review of the literature and a report of two cases

Chronic kidney disease is an increasing public health problem, with a worldwide prevalence estimated to be between 8% and 16%. The metabolic alterations induce bone and soft tissue changes, and the encompassing term chronic kidney disease-mineral and bone disorder syndrome (CKD-MBD) is used to describe them. The cardinal manifestations of the syndrome are bone catabolism and soft tissue calcifications, which ultimately compromise the cardiovascular and skeletal systems. In rare cases, tumorous enlargement of the craniofacial bones occurs. This article provides a brief review of the pathogenesis and imaging of craniofacial changes in CKD-MBD and reports on two cases of expansive jaw lesions. The term expansive renal osteitis fibrosa is recommended to describe these lesions.

Sequestrating Giant Complex Odontoma: A Case Report and Review of the Literature

Odontomas are the most common benign tumours of odontogenic origin. Due to their hamartomatous nature, they are usually asymptomatic but can cause impaction of one or more teeth. They consist microscopically of all the tissue types found in a developed tooth. We present a case of a large sequestrating complex odontoma resulting in facial asymmetry, cellulitis, pain and partial loss of function. This case has significance, as odontomas of this large size have rarely been reported.

Chronic kidney disease–mineral bone disorder: an update on the pathology and cranial manifestations

Chronic kidney disease-mineral bone disorder (CKD-MBD) is a syndrome encompassing skeletal and extra skeletal changes associated with chronic kidney disease. It progresses silently until an advanced clinical stage when complications impact on the quality of life and survival rates of patients. The maxillofacial manifestations are unique and may play an important role in the early identification of changes which could influence the management of these patients. The goal of this review is to highlight the maxillofacial features, pathology, and principles of management of CKD-MBD.

Recent Developments in Metabolic Bone Diseases: a Gnathic Perspective

Metabolic bone diseases often are asymptomatic and progress sub clinically. Many patients present at a late stage with catastrophic skeletal and extra skeletal complications. In this article, we provide an overview of normal bone remodeling and a synopsis of recent developments in the following conditions: osteoporosis, rickets/osteomalacia, endocrine-induced bone disease, chronic kidney disease-mineral bone disorder and Paget’s disease of bone. Our discussion will emphasize the clinical and microscopic manifestations of these diseases in the jaws.

Regarding the Use of the Term “Cementum” in Pathologic Proliferations

Dental cementum is a layer of mineralized connective tissue deposited on the surface of a tooth. When the sheath of Hertwig dissolves during tooth root formation, induction of the formation of cementum follows after contact is established between perifollicular ectomesenchyme and the dentinal surface. In a strict biological sense, application of the term ‘dental cementum’ implies attachment of the mineralized tissue to a tooth surface and there are no other reproducible microscopic, radiological or biochemical criteria to define cementum. The hypocellular, lamellar, globular mineralized deposits not attached to root surfaces in pathological proliferations are haphazardly referred to as cementoid [1], cementumlike material and psammomatoid deposits [2], curvi-linear deposits [3] or cementicles [4]. Without much reasoning and to the distraught of students of the subject, the prefix “cementum” disappeared and reappeared several times over the past half century in World Health Organization (WHO) classification systems of gnathial and extra gnathial lesions. Although the prefix ‘cemento’ was omitted from ossifying fibroma and osseous dysplasia in the 2005 WHO classification [5], the principle is established in the introduction to the chapter on odontogenic and maxillofacial bone tumours of the 2017 WHO book to reintroduce ‘cementum’ as a preamble to the term ossifying fibroma [2]. Yet where the neoplasm is discussed a few pages further, “ossifying fibroma” appears without the recommended preamble. In the same publication, the term “psammomatoid” is used to label mineralized tissue described as “bearing some resemblance to dental cementum” in extragnathial juvenile ossifying fibromas [2]. Familial gigantiform cementoma (FGC) is the only “cementoma” that survived the terminological conundrum. Initially used by Agazi and Belloni in a case report in 1953 [6], FGC was retained in all the WHO manuals since 1971. Evidence which accumulated in the literature indicates that most cases reported subsequent to the identification of the entity are not familial [7], small lesions in the early stage of growth can barely be described as “gigantiform” and whether the mineralized tissue is indeed cementum, is open to debate as the deposits are unattached to a tooth surface (Fig. 1). The microscopic appearance of FGC’s corresponds to conventional (cemento-) osseous dysplasias (OD’s). Both groups are characterized by a non neoplastic fibro-osseous proliferation. The mineralized component matures at the expense of the fibrous component passing through an immature radiolucent stage, followed by an intermediate mixed stage and ultimately the mature radiodense stage, when growth ceases. The signature macroscopic and radiologic feature of mature FGC and OD is conveniently described as ginger root-like masses and microscopic examination shows globular mineralized masses with minimal fibrous interstitial tissue. Unlike conventional OD’s, FGC’s involves younger patients and have an extended growth phase, which manifests clinically with gross expansion in the protracted early radiolucent stage [7]. Despite the overlap in their microscopic appearances and tendency for the mineral component to mature, FGC is not classified with the more common types of OD. Although a classification should be encompassing, rare variants of FGC’s with systemic abnormalities such as the calcium depletion disorder [8] and bone fragility [9] are not accounted for in any classification system to date. The statement that the microscopic features of FGC’s are ‘analogous to ossifying fibroma’ [2] (a neoplastic growth which does not mature), is confusing and justifies incorrect microscopic interpretations, of which there are several examples in the literature [10]. The term ‘periapical (cemento-) OD’ was inherited from the 1971 WHO classification where it was categorized with * Erich Raubenheimer raubenheimere@ampath.co.za; ejraub@fox5.co.za

Primary extranodal Hodgkin's lymphoma of the faucial tonsil

Primary extranodal involvement of Waldeyer′s ring by Hodgkin′s lymphoma (HL) is a rare occurrence and only isolated cases have been described. The aims of the study were to review primary extranodal HL of the tonsillar region and to present a new case without evidence of systemic involvement. The differential diagnoses, immunologic findings, and features of these rare tumors are discussed. All manuscripts published on primary extranodal HL of the pharynx were retrieved from the literature, using PubMed as a search engine, and the data compared with a case diagnosed in a 64-year-old male. Although a diagnosis of HL is not considered generally in the differential diagnosis of the pharyngeal mass, histopathologists should be aware of its possible occurrence.