Osteosclerotic plasmacytoma of maxillary bone (orbital floor)

Plasma cell neoplasms have been classified as multiple myeloma, solitary plasmacytoma and extramedullary plasmacytoma. They are usually considered as osteolytic lesions of bone except for the rare occurrence of osteosclerotic lesions. This paper describes the first reported osteosclerotic plasmacytoma of the maxillary bone and orbital floor. The difficulties in establishing a diagnosis and the relationship to other plasma cell neoplasms are discussed. Osteosclerotic plasmacytomas are a rare variant of plasma cell tumors which usually produce osteolytic lesions rather than bony sclerosis. Sixty-eight patients with the osteosclerotic variant have appeared in the world literature, with an overall incidence of about 1 per cent in a large series of plasma cell neoplasms (Dreidger and Pruzanski, 1979). There have been only six previous cases of solitary osteosclerotic plasmacytomas reported (Morley and Schweiger, 1964; Roberts et al., 1974; Rodriguez et al. 1976; Rushton, 1965; Schneinker, 1938; Brigham Medical Review, 1961) involving spine, sternum, or rib. None have previously been reported in the head and neck area. Plasma cell tumors have been classified into multiple myeloma, solitary plasmacytomas of bone, and extramedullary plasmacytomas. Multiple myeloma is a disseminated plasma cell malignancy characterized by the production of homogeneous immunoglobulins (whole or fragments) which appear in the serum and urine. Plasma cell tumors can also occur as solitary plasmacytomas, usually in bone, but also in soft tissue. With time, most solitary plasmacytomas develop disseminated disease with all the characteristics of multiple myeloma (Wiltshaw, 1976). Extramedullary plasmacytomas arise in soft tissue rather than bone, and primarily occur in the head and neck region. Clinically, they remain localized and less frequently develop into disseminated myeloma.     

Distant metastasis in malignancies of the head and neck

It is a well known fact that disease in head and neck cancer remains confined above the clavicle in a majority of cases for a considerable length of time. The causes of death in head and neck cancer patients are known to be due to lymph node metastasis, fungation, asphyxia, cachexia, invasion of major vessels and infection. Distant metastasis occurs late in the disease. However, many reports have appeared in the literature which indicate a high incidence of distant metastasis in head and neck malignancies. Merino et al. (1977), in a clinical study, have indicated an incidence of 10.9 per cent. Studies based on autopsy findings, however, quote a much higher figure (30 per cent, Papac, 1984; 57 per cent, Gowen and Dessuto Nagy, 1963). Dennington and Caster (1980) reported that at least seven per cent of patients with head and neck cancer have distant metastasis when first seen. The present study was designed to find out the incidence of distant metastasis in our head and neck cancer patients.     

Non-Hodgkins lymphoma of the temporal bone

The temporal bone may be involved by primary or secondary neoplasms. The latter are uncommon but well documented in the literature, the usual primary sites being breast, kidney, lung, stomach, larynx and prostate (Schuknecht et al., 1968). Lymphoma rarely invades the temporal bone and is usually confined to the lymphoreticular system and gastrointestinal tract. A case of non-Hodgkins lymphoma of the temporal bone is presented.     

Plasmacytoid myoepithelioma of palate: three rare cases and literature review

Salivary gland myoepitheliomas are rare tumours, accounting for less than 1 per cent of neoplasms of the salivary glands. Myoepithelioma of the palate is very rare, and only a few cases have been reported in the world literature. Histological and immunohistochemical analysis supports the myoepithelial origin of this tumour. Here, we report three cases of myoepithelioma of the palate in adult males and review previously reported cases.     

Extraosseous osteogenic sarcoma of the parotid gland

The first reported case of an extraosseous osteogenic carcinoma of the parotid gland is presented. The head and neck region is an unusual site for these unusual neoplasms, with approximately 5 per cent of all extraosseous osteogenic sarcomas originating in the soft tissues of the face and neck. The neoplasms may arise after a latent period following radiation therapy. The majority, however, arise de novo. As a group, extraosseous osteogenic sarcomas are very aggressive and lethal neoplasms with an average 5-year survival of 15.6 per cent (Rao et al., 1978).     

Ossifying fibroma of the head and neck: Involvement of the temporal bone — an unusual and challenging site

Ossifying fibroma of the head and neck is most commonly described in the mandible and maxilla. A few isolated reports in the literature exhibit the rare existence of this lesion in the nasal bones, orbit, ethmoid sinus, sphenoid sinus, occiput, and in only two well-documented cases, the temporal bone. We present the case of an extensive ossifying fibroma of the temporal bone that presented as a suspected case of hypertosis of the external auditory canal and conductive hearing loss, without any cosmetic deformity. A review of the recent literature concerning the sites, radiologic presentation, difficulty of pathologic differentiation, and modality of therapy is discussed.     

Carcinoid tumour of the middle ear

Carcinoid tumours belong to the rarer neoplasms of the middle ear. In 1980, Murphy et al. described the first case of a carcinoid tumour of the middle ear. Only five cases could be traced in the literature. The light and electron microscope features of a primary atypical carcinoid tumour of the middle ear are presented and compared with those described in the literature. Primary carcinoid of the middle ear appears to be derived from the epithelial lining of the middle ear. Distant metastases have not been reported. The duration and nature of the symptoms are of little diagnostic value. An atypical carcinoid tumour in the left ear of a 33-year-old woman is described.     

Chondrosarcoma of the larynx: review of the literature and report of three cases

Three patients with laryngeal chondrosarcoma were observed at the ENT Department of Padua University from 1966 to 1983. Including the present observations, about 150 of these neoplasms have been described in the literature. The male to female ratio was 3:1, and the age at diagnosis ranged from 33 to 91 years (median, 66 years). The rate of metastasis was 8 per cent, and histologically positive lymph nodes developed in only five patients. Surgery is the treatment of choice for laryngeal chondrosarcoma. Radical neck dissection does not appear to be indicated, unless results of clinical examination suggest metastatic lymph nodal involvement.     

Malignant melanoma of the larynx

Malignant melanoma of the larynx is a rare tumour. Only 31 cases of primary laryngeal melanoma have been reported in the literature. Cady et al., (1968) found only four cases in a group of 2,500 cases of laryngeal cancer seen over a 25-year period. A case of primary malignant melanoma of the larynx is presented along with a review of the literature. Problems associated with histological diagnosis are discussed. This patient was regarded as a poor risk for laryngectomy and appears to have control of local disease following application of Carbon dioxide laser.     

Leiomyoma of the palate

Leiomyoma, a benign neoplasm of smooth muscle cell origin, has been documented infrequently in the oral cavity. It may arise wherever unstriated muscle is present including the arterial wall (Cherrick et al., 1973). The most common site is the uterus, followed by the wall of the alimentary canal and in subcutaneous tissue (McCaffrey et al., 1978). A search of the literature revealed 51 cases of oral leiomyomas. Cherrick et al. (1973) reviewed 35 cases, including seven of their own. Galili and Shteyer (1974) summarized the clinical data of 42 cases of oral leiomyomas reported since 1884. We can find only nine more reported cases of leiomyoma in the oral cavity: one each by Rhatigan and Kim (1976), Shirota et al. (1976), Kelly and Harrigan (1977), Schweigel (1980), Davis (1980) and Mechlin et al. (1980); and three cases by Damm and Neville (1979). The common sites in the oral cavity are the tongue and cheek but its occurrence on the palate is rare for out of the 51 cases of oral leiomyomas, only 10 involved the palate (one case each was reported by Fein, 1905; Kist and Bhaskar, 1964; Garrett, 1969; Galili and Shteyer, 1974; Rhatigan and Kim, 1976; and Davis, 1980; and four cases by Cherrick et al., 1973). Its rarity and large size make the tumour worth reporting.     

Aneurysmal bone cyst of the mandible

Aneurysmal bone cyst is an uncommon benign lesion that rarely presents in the craniofacial region. No prior reports of this entity involving the mandible could be found in the otolaryngologic literature, and it has been reported only infrequently in the maxilla. Two previously unreported cases originating in the mandible are presented with a review of the literature, pathology, and diagnosis of this lesion. Treatment of this lesion consists of complete surgical removal and immediate bone grafting for reconstruction.     

Hyperparathyroidism with osteitis fibrosa cystica in the maxilla

In hyperparathyroidism, skeletal changes occur in 13 per cent of severe and long-standing cases (Smith, 1979). These skeletal changes vary from generalized demineralization of bone in early cases to resorption of bone marrow and replacement by fibrous tissue with cystic changes, the latter being termed osteitis fibrosa cystica or brown tumour. These cystic tumours are rare and usually occur in the long bones. Osteitis fibrosa cystica rarely presents in the maxilla as the initial symptom of hyperparathyroidism.     

Malignant melanotic schwannoma of maxilla

Malignant schwannoma in the naso-maxillary region is very rare. Only 20 eases have been reported in the literature (Maheshwari et al 99) Melanotic variety of this tumor is extremely rare and only 4 cases have been found in internet search. Surgery followed by radiotherapy has been advocated for treatment. A case is reported involving maxilla in a 13 year old female patient because of its extreme rarity. The case was treated by surgery and showed no recurrence during a 3 year follow-up period.     

Giant-cell reparative granuloma of the temporal bone: a case report and review of the literature

Giant-cell reparative granuloma (GCRG) is an unusual, non-neoplastic fibrous lesion that most often arises in the mandible and maxilla. GCRG of the temporal bone is exceedingly rare. To the best of our knowledge, only 17 cases have been previously reported in the international medical literature. Although no case of metastasis has been reported, this malignancy can be locally aggressive, and it often recurs following incomplete excision. We report the case of a young woman with a very large GCRG of the right temporal bone. We discuss the clinical picture, differential diagnosis, histologic evaluation, appearance on computed tomography and magnetic resonance imaging, and treatment options. We also review the cases of temporal bone GCRG that have been reported in the literature so far.     

Malignant fibrous histiocytoma of maxilla — a report of five cases and review of literature

Malignant fibrous histiocytoma of the maxilla is a rare malignant tumor of the bone. Affection of maxilla does not occur very commonly. In this report five cases of fibrous histiocytoma have been presented along with a review of literature.     

Putative histogenesis of post nasal angiofibroma

Inspite of the histological resemblance, these tumefactions do not behave like true neoplasms. Harma (1959) believed their histogenesis from angioblasls. Taxy (1977) regards the fihroblasts as the main stroma cell which has resemblences like those of the granulation tissues, and hence appear to be hybrid fihroblasts. Vascular and fibrous element could be produced by any mesodermal “stem cell” (Shenoi. 1989). Therefore both fihroblasts and angioblasts may be inter-convertible. The cavernous element of tumour suggest it to be the hamartoma. The posterior part of nose and turbinates have very vascular and cavernous mucosa. The hamartomas are influenced by endocrine factors and growth hormones. The mesodermal element of this tumour also appear to have androgen receptors (Lee et al, 1980). These endocrine factors could be the initial triggering mechanisms, for their development appear most rapidly at the parapubertal age. The tumour is also influenced by peri-and apocrine factors. The eytogenic growth factors are locally available. The neoangiogenesit is, perhaps, influenced by these factors, which also induce destruction of basement membrane of vessels, migration and mitosis of angioblasts and formation of new blood vessels of capillary and larger sizes. The maturation of tumour appears to he heralded by reduction in endocrine factor tike growth hormone and establishment of sex hormones beyond the puberty. Further it is accelerated by local peri-and apocrine factors contributed by macrophages, lymphocytes etc. Those cases with heavy infiltration, therefore, have more often the signs of maturity like collagenisation, encapsulation, obliteration of vascular elements and necrobiotic phenomenon. These are exceptional tumours estntially observed amongst para-puberal males, having easy bleeding tendency and multidirectional extensions. They destroy the hones of skull and occupy adjoining cavities including cranial extensions in almost 20% cases (Ward et al, 1974). There is high recurrence rate (20 to 50% after initial excision). There are different views regarding their aetiopathogenesis, but none is able to explain all its features. These are considered to be inflammatory or allergic in origin (Willis, 1953), hyperplastic tissue reactions (Harma, 1959), due to androgen deficiency (Martin et al., 1948), vascular malformation (Osborn,1959) and hamartomas (Mishra & Bhatia.1964). Lately, micro-histological and marker techniques and better understanding of oncogenesis, have been available which give more insight in explaining its behaviour. In view of controversies, a retrospective study of more than 300 cases of angiofibromas is carried out to formulate a putative pathogenesis of this tumour and its peculiar behaviour.     

Revision stapes surgery

The records of 120 patients who had undergone revision stapedectomy were analyzed to determine: (i) the causes of failure; (ii) how to prevent failure by taking precautions during primary surgery; (iii) hearing results; and (iv) possible identifying factors which might pinpoint those patients with a high risk of sensorineural deafness. A review of these cases demonstrates that the results of revision stapedectomy are different from those of primary stapedectomy. The commonest cause of failure was prosthetic dislocation (30.8 per cent), followed by fibrous adhesions (18.3 per cent) and otosclerotic regrowth (14.1 per cent). First revision operations resulted in post-operative bone-air gaps of 15 dB. or less in 46.5 per cent of cases, much better than 25 per cent for second revisions. Primary stapedectomy resulted in successful closure of the air-bone gap to 15 dB. or less in 89.5 per cent of cases. The sensorineural loss occurred in 11.3 per cent or first revisions and in 16.6 per cent of second revisions, as compared to 1.3 per cent after primary surgery. 'Dead ears' were encountered in 2.2 per cent of first revisions, as compared to nil in the primary group.     

Melanoma of the nasal fossae and sinuses, apropos of a series of 7 cases

The authors present their experience of the malignant, mucosal melanomas involving the nasal cavity and paranasal sinuses through a retrospective study of 7 cases, observed in Rouen from 1977 to 1988. The results are compared to a review of world literature. The mucosal melanoma of the nose and sinus is a rare entity, representing approximatively 40 per cent of all sinonasal neoplasias. The signs and symptoms of sinonasal melanomas are non-specific. Therefore, the diagnosis is often delayed. The histological diagnosis is difficult given the substantial polymorphism of these tumors and the experience of achromatous forms. Electron microscope study and immuno-histo-chemical techniques using various monoclonal antibodies make it possible to eliminate the other major, differential diagnoses. Surgery is the primary method of treatment, sometimes combined with adjunctive postoperative radiotherapy. More recently, combined treatment with interferon and cimetidine appears to be interesting. The prognosis is very poor, if compared to skin melanomas, with a five year survival rate of 5 to 30 per cent. Local recurrence at the primary site and distant metastases are the most common caused of treatment failure.     

Subglottic haemangioma in children: experience with open surgical excision

Subglottic haemangioma is a potentially life-threatening condition for which various treatment modalities are available. The objective of this study was to evaluate our results for open excision of subglottic haemangioma. The study assessed 18 patients who had been treated at a paediatric tertiary referral centre. Most of these patients (83.3 per cent) had undergone open surgical excision without post-operative tracheostomy and had been intubated for several days post-operatively (single-stage procedure). In most of these patients (66.7 per cent), an anterior cartilage graft had been used for reconstruction. The average follow up in this study was 25 months. All the patients in this series had achieved an adequate airway after the procedure. One patient had developed a recurrence of haemangioma in the trachea at a later date. The results of open surgical excision in this study were very encouraging. Seventeen out of 18 (94.4 per cent) patients had avoided tracheostomy or had been decannulated as a direct result of surgery. One of these 18 patients (5.6 per cent) had required a temporary post-operative tracheostomy for 13 months as the subglottis cleared; this was classed as a partial success. Our experience is that open excision is a highly successful 'one stop' treatment for subglottic haemangioma, which avoids prolonged use of steroids and multiple endoscopic procedures. No patient in this series developed subglottic stenosis, which can be a significant complication of laser application.     

Salivary gland choristoma of the middle ear: case treated with KTP laser

Salivary gland choristoma of the middle ear is rare. It consists of non-malignant, non-growing, normal salivary gland tissue in the middle ear. It is a developmental abnormality that occurs around the proximal part of the second branchial arch before the fourth month of intrauterine life. The authors found the 25th recorded case in our centre and another 24 reported cases from a review of the literature between 1961 and 1999. Intratympanic salivary gland choristoma frequently occurs during the first and second decades of life and with a female preponderance (56 per cent). Nearly all the patients (96 per cent) in our review presented with a hearing loss, that had begun since birth, in infancy, or during childhood. Tinnitus (28 per cent), and serous otitis media (24 per cent) were also commonly present. One case complained of otorrhoea. Intratympanic and extratympanic anomalies were found in 96.2 per cent and 34.6 per cent of cases respectively. Of these anomalies, ossicular chain (88.5 per cent), facial nerve (65.4 per cent), middle-ear muscles (30.8 per cent) and labyrinthine windows (23 per cent) were the four most common sites. Therefore, salivary gland choristoma may represent a manifestation of a congenital ear anomaly. Diagnosis of salivary gland choristoma is generally not documented pre-operatively, but is based on surgical biopsy and histopathological investigations. Treatment of this rare lesion depends on the size, location and extent of the mass, degree of anatomical abnormality and expertise of the surgeon. In difficult cases where the mass is attached to the dehiscent or inferiorly placed facial nerve, only biopsy is recommended. However, complete surgical removal is advocated for a mass that is easy to remove. KTP laser use via a 200 micron fibre-optic light carrier can facilitate removal especially in cases with ossicular chain involvement.