Misplacement of dysplastic epithelium in Peutz-Jeghers Polyps: the ultimate diagnostic pitfall?

Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Small bowel polyps in the syndrome may exhibit epithelial misplacement, into the submucosa, the muscularis propria, and even the subserosa. The authors demonstrate two patients in whom there is also misplacement of dysplastic epithelium into the submucosa and muscularis propria of the small bowel. Epithelial misplacement is recognized to mimic invasive malignancy. Such mimicry is heightened substantially when the misplaced epithelium is dysplastic. Correct interpretation of the histologic changes is aided by the use of special stains, which demonstrate the associated lamina propria and the lack of a desmoplastic response, and immunohistochemistry, which shows that the misplaced dysplastic epithelium is accompanied by non-neoplastic mucosa. There is an increased prevalence of gastrointestinal malignancy in Peutz-Jeghers syndrome. However, the presence of perplexing histologic features, caused by epithelial misplacement, especially when some of that epithelium is dysplastic, in small bowel polyps at least has the potential for the overdiagnosis of malignancy in the syndrome.

Solitary fibrous tumour of the diaphragm.

Solitary fibrous tumours are uncommon neoplasms that have been described as occurring in the pleura, peritoneum, lung, mediastinum, pericardium, nose and paranasal sinuses. This report describes the first known case of a solitary fibrous tumour of the diaphragm, occurring in a 60 year old woman with a two year history of respiratory symptoms. The lesion was initially misdiagnosed as an elevated left hemidiaphragm. The operative findings and histological appearance of the tumour are described. A review of the literature examines the various types and presenting features of solitary fibrous tumours as well as their clinical behaviour and postulated origin.

Intranasal glomangioma.

Glomangioma is a benign tumour of the glomus body. It is a relatively rare tumour in the head and neck region and even rarer in the nasal cavity. We present the 13th documented case of an intranasal glomangioma. A 56-year-old woman presented with a lesion on the nasal septum associated with intermittent nasal pain and epistaxis. Local surgical excision was not only diagnostic but therapeutic as it effected a cure. Intranasal glomangioma has no well-defined presenting features but must be borne in mind as a possible rare cause of nasal pain and epistaxis. We also briefly review the clinical presentation, histology and management of such a rare tumour.

The histology of 'stored' autologous ossicles.

Controversy still exists concerning the ultimate fate of transposed or transplanted ossicles in tympanoplasty. Histological studies on autologous and homologous ossicles removed at revision operations, or from animal studies, vary in their conclusions. The viability, of such materials used in the long-term is still uncertain. Whether this has any functional significance is also uncertain. This report contains a histological review of 10 ossicle remnants stored in the 'closed' mastoid cavity for 12 months, following closed cavity mastoidectomy (combined approach tympanoplasty).

Efficacy and safety of large-loop excision of the transformation zone.

Large-loop excision of the transformation zone (LLETZ) has become a popular treatment for women with cervical intraepithelial neoplasia (CIN) before long-term effectiveness and safety have been fully evaluated. Women who took part in a 1990 study of the procedure have been followed-up by cervical cytology for longer than 2 years. The rate of recurrences and residual lesions was 5.0% in the first year and 0.6% in the second year. LLETZ was also effective when used as a repeat procedure, although the negative histology rate was much higher (4.7% for initial procedures and 20% for repeat procedures). 250 women from the original study group of 1000 answered a questionnaire on fertility and menstrual symptoms 3 years after LLETZ. We found no differences between these women and controls of the same age, living in the same geographical area, with a history of negative cervical smears. LLETZ is a safe and effective procedure with no effect on menstruation or fertility.

A fatal case of scleredema of Buschke.

Scleredema of Buschke is a rare disorder characterized by the development of areas of skin induration which usually resolve spontaneously. It is occasionally associated with a benign gammopathy, and rarely with myelomatosis. We describe a 60-year-old woman with extensive skin changes, who developed IgA myeloma. Unusually, her skin disease did not respond to conventional myeloma therapy. Death occurred as a consequence of the progressive skin disease.

Fat-wrapping in Crohn's disease: pathological basis and relevance to surgical practice.

The connective tissue changes that accompany intestinal Crohn's disease have received little attention from pathologists. This is particularly so with fat hypertrophy, and yet surgeons have long recognized the phenomenon of fat-wrapping in the intestines and used it to delineate the extent of active disease. A consecutive, unselected series of 27 intestinal resections performed on 25 patients for histologically confirmed Crohn's disease was studied to correlate fat-wrapping with other clinicopathological features. Fat-wrapping was identified in 12 of 16 ileal resections and in seven of 11 large bowel resections. It correlated closely with transmural inflammation and there was a relationship between fat-wrapping and other connective tissue changes including fibrosis, muscularization and stricture formation. Morphometry demonstrated that there was true hypertrophy and that fat-wrapping does not relate solely to bowel wall shrinkage. There was correlation with ulceration but in 11 cases macroscopic ulceration extended beyond the fat-wrapping and in six to surgical resection margins. The pathological features of 225 small intestinal resections were reviewed and fat-wrapping was seen only in Crohn's disease. Fat-wrapping correlates best with transmural inflammation and represents part of the connective tissue changes that accompany intestinal Crohn's disease. Findings also suggest that fat-wrapping alone should not be used as an accurate marker of disease extent at the time of surgery.