Lymphoma cutis with osseous involvement.
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Coronal computed tomography is an important adjunct to the routine axial view in the evaluation of lesions of the skull and brain in infants and children. Of 400 axial scans performed over a four-mounth period, 50 were supplemented by coronal scans. Intracranial or cranial abnormalities were present in 39 (78%). Although the disadvantages of the coronal projection make it impractical as the only modality, when used as an adjunct to the axial view certain lesions of the skull and brain are diagnosed and delineated better.
The Muir-Torre syndrome of sebaceous neoplasms of the skin, with or without keratoacanthomas, and multiple low-grade visceral malignancies with prolonged survival is a rare disorder. Colonic polyps are frequently present, and the syndrome appears to be familial. We report 2 unrelated patients with the Muir-Torre syndrome. Each case exhibited sebaceous adenomas. Gastrointestinal findings included colonic adenocarcinomas and a tubulovillous adenoma. Although an unusual disease, the Muir-Torre syndrome requires recognition because these patients are at risk for multiple primary malignancies and may have family members also at risk.
Sturge-Weber syndrome is a rare congenital disease which can impair a child's brain functions and psychosocial development. Its psychiatric aspects have been ignored in the literature. Two male Chinese patients who presented with paranoid disorders and one female Chinese patient who presented with depressive pseudo-dementia are described. Biological and psychosocial aspects of pathogenesis are discussed, especially in the light of a "shame-humiliation model" of paranoid processes, since shame and humiliation are engrained in Chinese culture. These case descriptions suggest an overlooked association of Sturge-Weber syndrome with psychopathology and have important implications for the prevention and treatment of such potentially crippling psychiatric complications in subjects with this syndrome as well as other visible physical handicaps.
Neurotropic melanoma is a lesion which simulates a cutaneous fibrous tumor whose clinical course is characterized by local infiltration, multiple recurrences, and commonly by metastasis. Like adenoid-cystic tumors of the head and neck, the neurotropic melanoma has a predilection for perineural invasion and extension along peripheral nerves. Its microscopic picture is characterized by atypical "neuroma-like" patterns of Schwann cells and spindle cells, by poorly defined margins, and by neurotropism. Lentigo malignant melanoma (LMM) is a precursory lesion. The neurotropic melanoma is of particular interest to the head and neck surgeon because in the less than 50 reported cases of this rare tumor the preponderant sites were head, neck, and lip. We report two cases of neurotropic melanoma of the head and neck which demonstrate the characteristic perineural invasion of cranial nerves and extension along cranial nerves to the central nervous system. The clinical and pathologic features of the neurotropic melanoma are reviewed and the key diagnostic and therapeutic considerations in these patients are discussed.
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The case of a 54-year-old man with multiple basaliomas of the face but without any other symptoms of the Goltz-Gorlin syndrome is described. Obviously, there ist a family history of a tendency to develop basaliomas. One of the patient's three brothers, his mother, her sister and two of his six children were also affected. After removal of the tumor, electron microscopy revealed intranuclear crystal-like structures in some of the epithelial cells of the tumor tissue. The particular geometry of these structures suggests that these are papilloma viruses. The tumor stroma contained fibroblasts, fibrocytes and predominantly myofibroblasts. Tumor explants were cultured in vitro in Dulbecco-Vogt medium using 5 different supplements: 10% fetal calf serum (FCS), 10% fresh human serum with and without PDGF and CL (PDGF-CL, PDGF, CL, NULL). These cultures result in different rates of collagen gel contraction and binding of varying amounts of the "q" fraction of the first complement (C1q): PDGF CL-cells greater than PDGF cells greater than FCS cells greater than NULL cells. The culture of tumor tissue cells in different media thus seems to enhance the dominance of different cell subtypes.
Rhabdomyosarcoma of the head and neck often presents with vague symptoms which mimic other disease conditions. These factors lead to undue delay in the establishment of the correct diagnosis and the delivery of acceptable therapy, including surgery, radiation therapy, and chemotherapy. There is, however, evidence of improved results of treatment of these tumors since the addition of multiple drug chemotherapy to surgery and radiotherapy.
Intraoperative facial nerve monitoring provides a potentially useful adjunct to recent surgical advances in neurotology and neurosurgery. These measures further aid the surgeon in preserving facial nerve function by enhancing visual identification with electrical monitoring of mechanically evoked facial muscle activation. Facial nerve monitoring in neurotologic surgery may achieve the following goals: (1) early recognition of surgical trauma to the facial nerve, with immediate feedback made available to the surgeon through monitoring of mechanical activation; (2) assistance in distinguishing the facial nerve from regional cranial nerves and from adjacent soft tissue and tumor with selective electrical stimulation; (3) facilitation of tumor excision by electrical mapping of portions of tumor that are remote from the facial nerve; (4) confirmation of nerve stimulability at the completion of surgery; and (5) identification of the site and degree of neural dysfunction in patients undergoing nerve exploration for suspected facial nerve neoplasm or undergoing decompression in acute facial palsy. This paper provides an overview of intraoperative facial nerve monitoring principles and methodology and reports a recent clinical investigation that demonstrates the utility of facial nerve monitoring in translabyrinthine acoustic neuroma surgery.
Significant advances are being made in the treatment of osteosarcoma and improvement in the five year survival is being reported. Active study of the etiology, pathogenesis, immunology, immunotherapy, radiotherapy, chemotherapy, and surgical management is currently in progress. The preliminary survival statistics with adjuvant chemotherapy or immunotherapy are encouraging and it is hoped that these survival figures will be maintained with further follow-up. Our experience suggests that, when practical, patients with osteosarcoma should be treated in or in cooperation with centers actively involved in the evaluation of osteosarcoma therapy. Not only does this step offer the patient optimal opportunity to receive the most current therapy, but it is essential in obtaining adequate numbers of patients for critical analysis.
The calcifying epithelioma (pilomatrixoma) is a benign tumour of the skin originating from hair sheath cells. The head, especially the cheek and preauricular region with the parotid gland, is a very common site (about 50%). The tumour arises predominantly in younger people, with a 2:1 female to male ratio. It is not so rare as suggested by the few ENT publications. The histologic picture shows two characteristic epithelial cells, the basophilic and ghost cell. Calcification is present in most cases. The prognosis after removal of the tumour is good. Two personal cases are reported, multiple pilomatrixomas on the cheek of an 12 year old girl, and a tumour on the auricle, an unusual site.
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