Recurrent melanotic neuroectodermal tumour of infancy. Case report and survey of 16 cases

A case of melanotic neuroectodermal tumour of infancy (MNTI) with rapid recurrence is described, together with a survey of 16 reported recurrent cases. The recurrent lesion was successfully treated by wide excision of the maxilla. The recurrent tumour had two lesions of different natures, one consisting of a lobular mass indistinguishable from the primary tumour but located remote from the original site and the other showing diffuse infiltration of tumour cells deep in the medulla of the maxilla. A survey of the recurrent cases indicated that, although incomplete removal of the primary tumour, dissemination of tumour cells during surgery and a multicentric origin could be the causes of a recurrence, the presence of a silent lesion which may express an aggressive growth on stimulation may also explain the complex behaviour of the tumour.     

Merkel cell carcinoma

Merkel cell carcinoma (Mcc) is a very rare, malignant, neuroendocrine tumour. Mcc has an aggressive behavior, local recurrence, and regional or distant metastasis generally develop within a short period of time. At the Oral Medicine Department 158,056 patients were treated between 1970 and 2004. A single case of Mcc was diagnosed, in a 79-year-old woman. The tumour was localized to the upper lip. After successful cryosurgery and a 7-year tumour-free period, a new tumour developed in her palatine tonsil. It was an anaplastic carcinoma with neuroendocrine features, raising the possibility of a late haematogenous metastasis, a second field tumour, or a second primary tumour. The clinical, histological, immunohistochemical and genetic findings suggested that the tumour of the palatine tonsil is a second field tumour.     

An ectomesenchymal chondromyxoid tumour on the lateral border of the tongue

Ectomesenchymal chondromyxoid tumour (ECT) is an extremely rare intraoral mesenchymal tumour. Most of these tumours have been identified on the anterior aspect of the dorsal surface of the tongue. ECT is difficult to diagnose because of its rarity. We report a case of ECT arising on the lateral border of the tongue in a 67-year-old woman. The tumour, measuring 20 × 10 mm in size, was surgically removed. Histopathologically, the tumour was composed of small polygonal cells arranged in sheets, with a myxoid or hyalinized stroma. The tumour boundary was clear; however, the tumour showed a multinodular structure expanding along the tongue surface without obvious capsule. Careful examination revealed the tumour nodule to be spreading in a skip lesion-like fashion away from the main part of the tumour in the striated muscle layer. Although there was no evidence of recurrence at 18 months after the surgery, our observations suggest that surgery for ECT resection with a safety margin is more appropriate than enucleation.     

Peripheral squamous odontogenic tumour

Squamous odontogenic tumour (SOT) is a rare, benign epithelial tumour of odontogenic origin. A recurrent case of SOT with peripheral occurrence is described. In 1971, a tumour from the anterior maxillary palatal mucosa of an 11-year-old female was excised. In 1984, when the patient was 24 years old, a recurrent tumour was found in the same area. The histopathological appearance of the lesion, which was found to be identical with the primary tumour, was that of an SOT. Both the clinical and radiographic features supported the extra-osseous involvement of both tumours. The SOT is further evaluated and its origin is discussed.     

Mandibular metastasis of nephroblastoma: a rare case

Wilms' tumour (nephroblastoma) is a renal tumour that develops in childhood; it is one of the most common malignant tumours in children under 5 years old. Bone metastases are extremely rare in Wilms' tumour. Reported here is a patient who underwent nephrectomy for Wilms' tumour at the age of 2 years and presented with a jawbone mass in her left mandible 9 months later.     

The tumour microenvironment: a novel target for cancer therapy

Cancer therapy is in the midst of a major paradigm shift. Traditionally, cancer treatments have focused on tumour cells. However, studies over the past few decades have demonstrated that cancer is a vastly complex entity with multiple components affecting a tumour's growth, invasion and metastasis. These components, collectively termed the 'tumour microenvironment', include endothelial cells, pericytes, fibroblasts, inflammatory cells, leucocytes and elements of the extracellular matrix (ECM). Biological agents that target components of the tumour microenvironment may provide an interesting alternative to traditional tumour cell-directed therapy. Because of the complexity of the tumour milieu, the most beneficial therapy will likely involve the combination of one or more agents directed at this new target. This review highlights recent preclinical and clinical studies involving agents that target tumour vasculature, leucocytes, pericytes, cancer-associated fibroblasts and ECM components. We pay particular attention to combination therapies targeting multiple components of the tumour microenvironment, and aim to demonstrate that this strategy holds promise for the future of cancer treatment.     

Extranodal extension in oral cancer: A role for the nodal microenvironment?

Oral squamous cell carcinoma (OSCC) is a significant cause of morbidity and mortality worldwide and accounts for the majority of head and neck cancers. Metastasis of primary tumours, primarily to cervical lymph nodes in the neck, is associated with worsening prognosis. Furthermore, the prognosis of patients with extranodal extension of metastatic tumour from the lymph nodes into the neck tissues is particularly poor. The factors affecting this process are poorly understood, and detection is difficult pre‐surgery. Mounting evidence shows that components of the tumour microenvironment including cancer‐associated fibroblasts, vascular and lymphatic endothelial cells, the extracellular matrix and inflammatory immune cells, are important modulators of tumour behaviour in primary OSCC and other cancers. However, little is known about the lymph node microenvironment, its response to tumour presence and role in extranodal extension. In addition, there are many lymph node‐specific cell types and structures, such as fibroblast reticular cells and high endothelial venules, making the lymph node microenvironment distinct from that found at primary tumour sites, and which contribute to the nodal response to tumour presence. This review details the current knowledge regarding the lymph node tumour microenvironment in OSCC and its role in lymph node metastasis and extranodal extension and relates this to features of the primary tumour. Understanding the role that the lymph node microenvironment plays in promoting tumour development and extranodal extension may aid the identification of novel biomarkers and alternative treatment strategies to improve the prognosis of patients with advanced OSCC.     

Nerve sheath myxoma of the tongue in a child

Nerve sheath myxoma (NSM) is a benign peripheral nervous system tumour that rarely occurs in the oral cavity. Among 17 cases of oral NSM described in the literature (average patient age 33 years), only two, including the present case, have been reported in children. The present case occurring in an 8-year-old boy was therefore extremely rare. Histopathologically, the tumour was found as multinodules under the mucosal epithelium, and was composed of spindle- or stellate-shaped cells with a myxoid background that stained with alcian blue and toluidine blue. Immunohistochemically, the tumour cells were strongly positive for S-100 beta protein and neuron-specific enolase. These results suggested that the tumour originated from Schwann cells.     

Expression of type 2 nitric oxide synthase and p53 in Warthin''s tumour of the parotid

Although the pathogenesis of Warthin's tumour is not fully understood, it is generally thought that the tumour arises from heterotopic salivary ducts within pre-existing lymphoid tissue. Prolonged nitric oxide (NO) production by the enzyme type 2 nitric oxide synthase (NOS2) has been implicated in the pathogenesis of many solid tumours, but not in Warthin's tumour. Since NO and NOS2 are known to be associated with p53, the immunohistochemical expression of both NOS2 and p53 was investigated in 23 cases of Warthin's tumour. Widespread diffuse cytoplasmic immunostaining for NOS2 was found in tumour epithelial cells of all 23 cases studied, and it was additionally expressed in normal salivary duct epithelium. p53 staining was localised to the nuclei of tumour epithelium in 16 cases, with a similar pattern of distribution to tumour NOS2 expression. A significant correlation was found between NOS2 and p53 staining in the tumours (P < 0.001). In contrast to NOS2, p53 was not expressed by normal salivary ductal cells in any of the cases studied. NOS2 is widely expressed by the tumour epithelium of Warthin's, and its association with p53 expression is discussed. The role of NO in the pathogenesis of Warthin's tumour remains to be established.     

Simultaneous adenomatoid odontogenic and keratocystic odontogenic tumours in a patient with Gorlin‐Goltz syndrome

Gorlin and Goltz described a syndrome in which multiple basal cell carcinomas, odontogenic keratocysts and bifid ribs occurred in combination. The jaw keratocysts are a consistent feature of ‘Gorlin‐Goltz’ or naevoid basal cell carcinoma syndrome. Central nervous system and ocular involvement occurred together with the fairly typical facial features of frontal bossing and hypertelorism. This case report documents the pathology associated with an impacted maxillary canine tooth in a boy with Gorlin‐Goltz syndrome. The patient presented for investigation of the failure of eruption of the right permanent maxillary canine tooth. Radiographic investigation showed the presence of a well circumscribed radiolucency located around the crown of an impacted right maxillary canine tooth. The patient's medical history revealed a medulloblastoma that was treated 13 years ago. The right maxillary canine tooth and associated peri‐coronal tissue were removed under general anaesthetic. A diagnosis of a keratocystic odontogenic tumour with an associated adenomatoid odontogenic tumour was made. The common differential diagnoses for a peri‐coronal radiolucency in the maxilla that need to be considered by dentists include a dentigerous cyst, follicular keratocystic odontogenic tumour and adenomatoid odontogenic tumour. A rare case of both keratocystic odontogenic tumour and associated follicular adenomatoid odontogenic tumour is described in a patient with naevoid basal cell carcinoma syndrome.     

Sialolipoma of the parotid gland.

A case of slow-enlarging mass of the parotid region in a 44-year-old male is presented, which proved to be a lipomatous tumour of the parotid gland. The clinical impression was that of a benign salivary gland tumour. The tumour was situated in the deep lobe of the gland, thus a total parotidectomy was performed, with preservation of the facial nerve. Microscopically the lesion was described as consisting of mature adipose tissue, which, however, encompassed both glandular elements and nerve bundles within it. This tumour has been recently recognized as a separate entity under the term 'sialolipoma'.     

Angiopoietin-2 inhibits the growth of tongue carcinoma without affecting expression of vascular endothelial growth factor

Angiopoietin-2 (Ang-2) has been identified as an important factor in tumour angiogenesis through its action in blocking the stabilizing actions of Ang-2 and leading to new tumour vessel growth in the presence of vascular endothelial growth factor (VEGF). In the authors’ previous study, over-expression of Ang-2 in Tca8113 tongue tumour cells inhibited growth. The current study aims to clarify the mechanisms of Ang-2-mediated tumour growth inhibition and its role in the regulation of VEGF expression. These studies used tumours derived from Ang-2-transfected Tca8113 cells injected into nude mice. The results showed that Ang-2-transfected tumours displayed aberrant angiogenic vessels with few associated smooth muscle cells. No detectable differences in VEGF expression were observed between Ang-2-transfected and parental tumours. Tumours produced by the Ang-2 transfection also had a higher apoptosis index and lower tumour cell proliferative index than tumours in the control groups. These observations suggest that enhanced expression of Ang-2 has no effect on VEGF expression and results in tumour vessel regression and inhibition of tumour growth.     

Central calcifying epithelial odontogenic tumour in the posterior maxilla: a case report

The calcifying epithelial odontogenic tumour (CEOT), or Pindborg tumour, is a rare, benign odontogenic tumour. CEOT is usually asymptomatic and an incidental radiological finding, often presenting as a mandibular radiolucency with flecks of calcific material. We report an unusual case of CEOT in the left posterior maxilla of a 46‐year‐old male that was associated with an unerupted tooth. The tumour in this case caused non‐specific sinus symptoms and appeared radiographically similar to an odontoma or ossifying fibroma due to its dense calcific contents. Diagnosis was confirmed histologically following surgical removal of the lesion, which showed classic CEOT histomorphology. We report this case to highlight the unusual clinico‐radiologic presentation and illustrate the diagnostic difficulties that can occur with radiolucent and/or radiopaque lesions in the jaws.     

Aggressive maxillary squamous odontogenic tumour in a child: histological dilemma and adaptative surgical behaviour

A case of a maxillary osteolytic tumour is described in a 9-year-old boy. Histological analysis led to an initial diagnosis of benign squamous odontogenic tumour, although this was not straightforward due to swelling, and cellular pseudo-malignant and non-specific signs. Because of the young age of the patient, a local surgical tumourectomy was first chosen with respect to the mixed dentition. For 10 months, the evolution was satisfactory. Then, a very aggressive tumoural recurrence with lip and palate infiltration led to doubts as to the histologic nature of the tumour. Efficient collaboration between several specialized pathologist teams finally confirmed that this was a squamous odontogenic tumour but in a very aggressive form. Radical surgery was then carried out.     

A desmoplastic granular cell tumour of the oral cavity: report of a case.

A case of granular cell tumour of the buccal mucosa demonstrating marked desmoplasia is presented. The bulk of the tumour is composed of dense collagenous stroma, which surrounds isolated nests of tumour cells. Since the histomorphologic appearance of this variant is different from that usually seen in this lesion, the diagnosis is supported by immunohistochemical demonstration of S-100 and Vimentin in the tumour cells. The authors believe that this case represents the first report of a desmoplastic granular cell tumour of the oral tissues.     

Can recurrence of giant cell tumour occur in an allograft? A report of two cases

Giant cell tumour is a locally aggressive tumour with a high incidence of local recurrence. The choice of treatment remains to be controversial. These tumours are usually managed with extended curettage in the early stage and with resection and replacements in the advance stages. This is an illustration of two case of Giant cell tumour, one of the proximal tibia and another of the distal radius that was treated with wide resection and osteoarticular allograft. Both patients developed recurrence of the disease within the allograft.     

Tumour of the juxtaoral organ

The juxtaoral organ is a normal and constant structure of the oral cavity. It consists of benign epithelial nests. We describe an intraoral tumour of the juxtaoral organ in a child. The tumour was not diagnosed after clinical and radiological examinations because it is extremely rare. A histological examination revealed a tumour of the juxtaoral organ, presumed to be neuroid hamartoma. This is only the second time that a tumour of the juxtaoral organ has been described in a child. We also describe the location, the embryology, the histology and the function of this organ. This is important because this structure can be confused with carcinomas of the oral cavity when examining frozen sections.     

Management of the parotid pleomorphic adenoma, the problem of exposing tumour tissue at operation. The logical pursuit of treatment policies

The objective of treatment of a parotid pleomorphic adenoma is to remove all tumour cells with minimal short- and long-term morbidity and minimal recurrence rates. If an enucleation is carried out, the facial nerve may be put at risk and tumour fragments will inevitably be left in the wound. Most surgeons suggest that after enucleation, radiotherapy must be given to reduce the recurrence rate to an acceptable level. If during a formal superficial parotidectomy the tumour capsule is visualised, the objective of removing the tumour with an intact cuff of normal tissue has failed and the operation has in effect become an enucleation. This paper defines the problem and discusses its management.     

鱼精蛋白抑制肿瘤生长的实验研究

目的:探讨鱼精蛋白抑制肿瘤生长的治疗机制,为其临床应用提供实验依据。方法:通过建立动物肿瘤模型,实验治疗,结合免疫组化及原位DNA缺口末端标记法,检测肿瘤内血管密度、肿瘤细胞增殖指数和凋亡指数。结果:治疗组肿瘤内血管密度和增殖细胞指数分别为9.52±3.76条/mm2和31.02±7.83%,均明显低于对照组(p<0.05),凋亡指数为7.67±1.70%,较对照组增高近一倍(p<0.05)。结论:鱼精蛋白确有抑制肿瘤血管生成的作用,同时限制了肿瘤细胞的增殖,并且能诱导其凋亡。其抑制肿瘤生长的治疗作用来自抑制血管生成、限制肿瘤细胞增殖和诱导细胞凋亡的共同作用。     

Maxillary fibrosarcoma with extracellular immuno-characterization

Maxillary fibrosarcoma of a 31-year-old female is described with special reference to immunofluorescent characterization of the extracellular matrix. Following extractions, endodontic treatment and occlusal adjustment the tumour was operated on. The spindle-shaped tumour cells displayed a herring-bone pattern. The tumour was diagnosed as a fibrosarcoma and graded as poorly differentiated. Immunostaining for the specific type I collagen was positive throughout the tumour matrix, whereas staining for type III collagen was faint and occasional, and did not definitely codistribute with reticulin. Staining for type IV collagen and fibronectin in the tumour matrix was negative.