More than meets the eye.

We report an extremely rare case of a hybrid tumour of the maxillary sinus. A 51-year-old man presented with a 6-week history of nasal congestion and epiphora. Radiological imaging demonstrated a maxillary sinus tumour, with extensive local invasion. Surgical excision included maxillectomy, left eye exenteration and free flap closure. Histology of the excised specimen showed a rare hybrid tumour containing adenoid cystic carcinoma, salivary duct carcinoma, epithelial-myoepithelial carcinoma and basal cell adenoma. Hybrid tumours are very rare tumour entities which are composed of at least two distinct tumour types. Each tumour entity conforms with a defined tumour type. The tumour entities of a hybrid tumour are not separated but have an identical origin within a definite topographical area. Diagnosis and appropriate management requires high index of suspicion, pathological endeavour to look for a more aggressive accompanying tumour and adequate oncological treatment according to the highest grade of tumour.

Conservative management of multiple keratocystic odontogenic tumours in a child with Gorlin-Goltz syndrome: a case report.

BACKGROUND: The recommendations regarding the management of keratocystic odontogenic tumour (KCOT) vary widely in the literature. The authors highlight that conservative surgical management should still be considered in some cases. CASE REPORT: A young patient with Gorlin-Goltz Syndrome and two large mandibular KCOTs is presented. The case demonstrates conservative treatment with enucleation of the tumours and preservation of all involved teeth. Subsequent orthodontic treatment resulted in all teeth erupting successfully. The tumour cavities regenerated with bone.

Prospective screening for multiple tumors of the upper aerodigestive tract: a simple routine procedure.

Detection of a second primary at the time of initial work-up of patients with head and neck cancers has an important bearing on management planning. In a 12-month period, a series of 105 patients with an index head and neck primary (skin malignancies excluded) of the squamous cell type were subjected to the following screening procedure. Under topical anesthesia, with the patient in the sitting position, a small-size flexible bronchofiberscope (Olympus B3R) was introduced through the nose. As the scope was advanced, the nasal cavity, nasopharynx, oropharynx, hypopharynx, and larynx were examined. The tracheobronchial tree was also examined if the chest x-ray showed abnormal findings. The endoscope was then passed down to examine the esophagus, with oxygen insufflated via the biopsy channel at a rate of 1 liter per minute. The index primary sites included the tongue (20), alveolus (7), floor of mouth (2), palate (4), buccal mucosa (3), hypopharynx (17), and larynx (52). There was little patient discomfort and no complication. Nine patients (8.5%) were found to have simultaneous primary tumors including two patients with triple primaries and one with quadruple lesions. The additional tumors were in the following sites: esophagus (6), tonsil (2), floor of mouth (1), nasopharynx (1), bronchus (1), palate (1), and oropharynx (1). The treatment plans were changed in all nine patients with the discovery of multiple tumors. It is concluded that the panendoscopic screening in the manner described is simple, safe, and convenient and the information is contributory to treatment planning.

Treatment of stage I carcinoma of the anterior floor of the mouth.

A retrospective study of 27 patients treated for stage I squamous cell carcinoma of the floor of the mouth was performed to assess the effectiveness of therapy. Of these patients, 23 (85%) underwent surgical therapy. Four patients (15%) were treated with radiation therapy alone, and three (11%) underwent combined surgery and radiation treatment. Initial treatment failed in seven patients (26%), and further therapy was curative in only four of seven. This small study suggests that stage I floor-of-the-mouth squamous cell carcinoma is not an easy lesion to cure. Other studies support these data. A three-dimensional monobloc resection with a 2-cm margin, including resection of the submandibular glands, is advocated. Toluidine blue O mucosal staining and Mohs' chemosurgery are helpful adjuncts in determining the tumor margins.

Neostigmine and polyethylene glycol electrolyte solution for the therapy of acute hepatic encephalopathy with liver cirrhosis and ascites.

We treated a 75-year-old man who had non-B and non-C, and Child's class C liver cirrhosis and acute hepatic encephalopathy with neostigmine and polyethylene glycol electrolyte solution. He received repeated transcatheter artrial embolization and percutaneous ethanol injection combination therapy for multiple hepatocellular carcinomas, which controlled his disease for 25 months from the first treatment. He was admitted in a state of hepatic coma after being found unresponsive at his home. With the consent of the patient's family, we gave him 1.0 mg of neostigmine intramuscularly to improve his peristaltic movement, and 2 L of polyethylene glycol electrolyte solution through a nasogastric tube for 4 hours to reduce the production and absorption of gut-derived toxins of nitrogenous compounds. Using these treatments, the blood ammonia level decreased to the normal range within 8 hours, and the coma disappeared after 2 days. We suggest that a combination approach of neostigmime and polyethylene glycol electrolyte solution may be one of the most effective treatments for acute hepatic encephalopathy associated with liver cirrhosis and ascites.

[Gorlin-Goltz syndrome with odontogenic keratosis. Report on a patient followed for 10 years]. / Sindrome di Gorlin-Goltz con cheratocisti odontogeniche. Presentazione di un paziente seguito per 10 anni.

A patient with Gorlin-Goltz syndrome (nevoid basal cell carcinoma syndrome) is described. This disease has an autosomal dominant inheritance pattern with complete penetrance and extremely variable expressivity. The case report seems to represent a new mutation. Gorlin-Goltz syndrome is characterized by a lot of symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. Also our patient showed recurrent jaw and maxillary cysts, for this reasons he has been followed for 10 years to the Oral Pathology Service of Galliera Hospital.

[Primary oral peripheral chondrosarcoma. Anatomo-clinical aspects and presentation of 2 cases with periodontal onset]. / Il condrosarcoma periferico orale primitivo. Aspetti anatomo-clinici e presentazione di due casi ad insorgenza parodontale.

After a brief analysis of the anatomoclinical aspects of chondrosarcoma in general and of the characteristic and particular aspects of the forms with maxillofacial onset (lower age of onset, elective sites of onset anterior in the maxillary and posterior in the mandible, often insignificant standard X-ray pictures, high percentage of error in clinical and histological diagnosis) two cases of peripheral periodontal onset, with low degree of malignity (grade 1), with aspecific clinical and radiological aspects, both locally recurrent several times after nonradical surgery are reported.

[Esophageal cancer and multiple primary cancer].

Two hundred eleven cases, 27.1%, of multiple primary cancers of esophagus and other organs were found in 778 cases of esophageal cancers which were treated in our institution. Among them, double cancer accounted for 92.9%, triple cancer accounted for 6.6% and quadruple cancer for 0.5%. As for the other organ of esophageal double cancer. 59.9% of them were head and neck, 25.1% were stomach, 4.9% were colon and rectum, and remaining included liver, breast, lymphoma lung kidney etc. Head and neck cancers consisted with hypopharynx, tongue, larynx, oral floor and gingiva regarding incidence in its order. For discovering of double cancer in esophagus and other organs, 1. head and neck, stomach, colon and rectum, lung, liver etc. should be investigated preoperatively in the patients of esophageal cancer, 2. Esophagus should be examined preoperatively in the patients of these cancers, 3. Screening of esophageal cancer should be performed in the patients of high risks of esophageal cancer. As for the multiple primary cancer of esophagus and other organs, the balance of treatment should be considered to take the priority of the cancer limiting the prognosis.

Severe florid cemento-osseous dysplasia: a case report treated conservatively and literature review.

BACKGROUND: Florid cemento-osseous dysplasia (FCOD) has been described as a condition that characteristically affects the jaws of middle-aged black women. Radiographically, FCOD appears as dense, lobulated masses, often symmetrically located in various regions of the jaws. FCOD is usually asymptomatic. In severe cases, focal expansion may occur due to infection. Management of the symptomatic patient is more difficult due to the avascular nature of the lesion which contributes to susceptibility severe infection, bone sequestration, and osteomyelitis when surgery is performed. CASE REPORT: This paper presents a rare case of severe FCOD; the black woman patient was diagnosed based on clinical and radiographic findings and treated conservatively. The examination of panoramic radiographs revealed a multiple sclerotic masses with radiolucent borders, found in the mandible and maxilla which were symmetrical at presentation. The patient continuous with the follow-up. DISCUSSION: This report confirms that a diagnosis can be made with accurate clinical and radiographic assessment. The correct selection of treatment for FCOD depends on this information.

Report of a rare and aggressive case of oral malignant melanoma.

BACKGROUND: Oral melanoma is a very rare malignancy with unknown etiology. Its higher incidence is between 41 and 60 years of age. A high localization of oral melanoma was found in the maxilla. Gender distribution was reported as 1:1. Oral melanoma represents 0.2% to 8% of the all the total cases of melanoma of the body. CASE REPORT: In this article we report an aggressive case of oral melanoma in a 71-year-old male with chief complaint of black swelling of the gingiva, paresthesia, and denture ill fitting. DISCUSSION: Most oral melanomas are asymptomatic and painless in early stages and unfortunately diagnosis delayed until symptoms occurred. Early detection of suspected melanotic lesions by patient, dentist, and physician is very important.

Small cell carcinoma ex-pleomorphic adenoma of the parotid gland.

Small cell carcinoma (SCC) is a high-grade malignancy usually encountered in the lungs but also seen in almost any site including the salivary glands. SCC is important to recognize because it often metastasizes widely and is treated with systemic chemotherapy. Carcinoma ex pleomorphic adenoma is a malignant epithelial neoplasm arising in a pre-existing benign mixed tumor (i.e., pleomorphic adenoma, PA). While virtually any salivary carcinoma can arise from a PA, to our knowledge SCC ex-PA has not been described. We report a case of a woman presenting with fullness of the right neck and a parotid gland mass. The tumor was resected and evaluated grossly, by light microscopy, and by immunohistochemistry. Grossly, a 1.6 cm well-circumscribed nodule was identified within the parotid. Microscopic examination revealed foci of SCC associated with high-grade adenocarcinoma, in the background of a PA. The SCC was immunoreactive for cytokeratin in a dot-like pattern and neuroendocrine markers synaptophysin and CD56. Despite the focal nature of the SCC in the parotid, a pure SCC metastasis was present in one neck level II lymph node. The patient was free of disease with 8 months of follow-up. Our case illustrates that: (1) although rare, in the salivary glands SCC may arise from lower grade neoplasms including PAs; (2) SCC ex PA may metastasize as pure SCC even if the primary SCC component was focal; (3) adequate sampling of PAs is crucial to prevent missing a rare SCC that must be treated with chemotherapy.

Bilateral metachronous osteosarcoma of the mandibular body: a case report.

Osteosarcoma of the jaw (JOS) is a rare phenomenon constituting 5% to 13% of all cases of osteosarcoma (OS). JOS has histological features similar to OS, but the biological behavior is different. The chief complaint of patients with long bone OS is pain, whereas patients with JOS usually have painless swelling as the first sign. OS may affect multiple sites. Multifocal or multicentric osteosarcoma is usually defined as metachronous (new tumors developing after initial treatment) or synchronous (multiple lesions at presentation) without pulmonary metastases. The incidence of this entity has been reported to be between 1% and 10% of all cases of OS. A 27-year-old man presented with bilateral metachronous osteosarcoma as painful swellings in the mandibular body. He was treated with a mandibulectomy and chemotherapy. Therefore, the physician should be aware that osteosarcomas can occur in different sites as true multicentric or metastatic lesions.

A patient death attributable to implant-related primary anaplastic large cell lymphoma of the breast.

UNLABELLED: Implant-related primary anaplastic large cell lymphoma (ALCL) of the breast is a rare clinical entity. With increasing attention being paid to this disease, most cases reported to date in the literature have demonstrated indolent clinical courses responsive to explantation, capsulectomy, chemotherapy, and/or radiotherapy. The authors describe a case of bilateral implant-related primary ALCL of the breast that proved refractory to both standard and aggressive interventions, ultimately resulting in patient death secondary to disease progression. The authors situate this case in the context of the current state of knowledge regarding implant-related primary ALCL of the breast and suggest that this entity is generally, but not universally, indolent in nature. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, V.

Intra-oral cancer at the Massachusetts General Hospital. Squamous cell carcinoma of the floor of the mouth.

A retrospective review of 163 consecutive patients with biopsy-proven, invasive squamous cell carcinoma of the floor of the mouth who underwent inpatient treatment at the Massachusetts General Hospital during the 15-year period from January 1962 through December 1976 is presented. The stage at first presentation, clinical features of the disease, incidence of second primary tumors, analysis of therapeutic modalities, and survival statistics are compared with reports from other large centers. Floor of mouth tumors comprised 28%, (163/592) of oral squamous cell carcinomas seen at the Massachusetts General Hospital during that time period. Seventy-one per cent of floor of mouth tumors were in men and 29% in women; women tended to present earlier in the course of their disease. Thirty-seven patients (23%) developed a secondary primary malignancy, and four of these 37 patients developed two second primaries. Distant metastatic disease appeared in 6% of patients with Stage I, II, or III disease and 26% of patients with Stage IV disease. Radiation therapy alone and surgery alone resulted in equivalent long-term survival rates for early stage disease. In more advanced stages (III and IV), a combined approach utilizing surgery and radiation therapy obtained superior results for short-term survival than either modality alone. The importance of early diagnosis and treatment and suggestions for development of cooperative protocols in an attempt to improve salvage of patients with this disease is discussed.

Oral condyloma acuminatum.

Fifty-one cases of oral condyloma acuminatum are reported, bringing the total number in the English medical/dental literature to approximately 156 cases. Ninety-five percent of the 59 new cases were in males. Eight-one percent occurred in the age range of 21-40 years. The most common locations were upper lip, lingual frenum, dorsum of the tongue, and lower lip. Thirty-four percent presented with multiple lesions.

[Acral ischemia as a rare paraneoplastic syndrome in the terminal phase of mouth floor carcinoma]. / Akrale Ischämie als seltene Paraneoplasie in der terminalen Erkrankungsphase eines Mundbodenkarzinoms.

BACKGROUND: The term "paraneoplastic syndrome" describes a clinically apparent disease associated with a malignant neoplasm, which is not a direct consequence of invasive tumor growth. Acral ischemia and digital necrosis of multiple fingertips have been occasionally described as a paraneoplastic syndrome. However, the exact mechanisms of this phenomenon are still not adequately known. CASE REPORT: This paper describes a 72-year-old male patient with severe acral ischemia and multiple necroses on several fingertips. Symptoms evolved under palliative chemotherapy with gemcitabine for inoperable metachronous squamous cell carcinoma of the tonsil following a history of two simultaneous carcinomas of the alveolar crest. Digital ischemia was combined with severe pain, similar to Raynaud's syndrome, which required therapeutic intervention. The treatment objective is to improve perfusion and simultaneously reduce pain. A combination of iloprost, an analog of prostacyclin (50 micrograms intravenous Ilomedin once daily), and a CT-guided thoracic sympathectomy achieved a continuous positive effect on the intensity of acral ischemia and alleviated the pain.