Johnson square procedure for lentigo maligna and lentigo maligna melanoma.

BACKGROUND: Lentigo maligna (LM) and lentigo maligna melanoma (LMM) can be difficult to manage surgically. Predetermined margins can be inadequate because of subclinical spread, or can affect function when margins are adjacent to the eye or mouth. AIM: To describe our 5-year experience in Nottingham of using the staged square procedure (Johnson square) in excising difficult facial LM and LMM. METHODS: The square procedure is a staged technique useful for ill-defined lesions and for lesions that have a high recurrence rate due to subclinical spread. It uses paraffin wax-embedded peripheral vertical sections for margin control, ensuring complete clearance as the surgical margins are usually examined at distances of 2-5 mm from the periphery of the lesion. RESULTS: We treated 21 patients with LM or LMM with the staged square procedure over a 5-year period. Of the 21 patients, 10 needed only one stage of surgery, 6 needed two stages, 3 needed three stages and 2 needed four stages. To date, there has been only one recurrence, which was of an extensive lesion that crossed the medial canthus, making margin control impossible because of the anatomical limitations. CONCLUSIONS: The staged square procedure is an effective treatment for LM and LMM. It attempts to conserve tissue while ensuring a higher clearance rate. This offers favourable cosmetic outcomes and better prognosis, especially for facial LM and LMM.

Acral histiocytic nodules: a possible new variant of non-X histiocytosis.

Many types of histiocytoses have been described. We present a case of a 56-year-old woman who presented with multiple nodules on the hands. On histological examination of an excision biopsy, a mainly dermal lesion was seen, with scattered mononuclear inflammatory cells and occasional multinucleated cells in the background. The tumour was composed of short spindle-shaped histiocyte-like cells with vesicular nuclei and small nucleoli. These cells were positive for CD68 and focally positive for smooth-muscle actin. There was no lipid or haemosiderin pigment, and no cholesterol clefts. Further investigations did not find evidence of bony or systemic involvement. The non-Langerhans cell histiocytoses, also known as non-X histiocytoses, are rare and of unknown aetiology. Clinicopathologically, this case does not seem to fit with any of the recognized subtypes, and we propose that it may represent a previously undescribed variant of the disease.

A novel case of follicular mucinosis after autologous stem-cell transplantation for multiple myeloma.

Follicular mucinosis is a rare inflammatory disorder of unknown aetiology, characterized by mucin deposition in hair follicles and sebaceous glands. FM can occur as a benign idiopathic primary disorder or secondary to malignant lymphoproliferative processes, most notably mycosis fungoides. We report a novel case of FM developing after autologous stem-cell transplantation for multiple myeloma, a correlation not previously reported in the literature.

Eccrine porocarcinoma.

We present an unusual case of eccrine porocarcinoma in that diagnosis was delayed for many years after the initial lesion appeared, resulting in relatively large tumor.

Hodgkin's disease in patients older than 70 years of age: a registry-based analysis.

Between 1973 and 1993, 529 patients aged 15 years and over with Hodgkin's disease (HD) were entered into a lymphoma registry. Twenty-eight cases (1 only diagnosed at autopsy) of histologically proven HD in patients aged 70 years or older were identified. The distribution of sex, 'B' symptoms, histology and stage was not significantly different from that of younger patients, except for the fact that there were no patients aged 70 years or older with lymphocyte predominant HD. Nineteen patients were treated radically, 5 patients palliatively and 4 patients received no radiotherapy or chemotherapy. Three of the 14 patients treated with chemotherapy achieved the planned dose intensity. The cause-specific 5-year survival was 75% for patients aged 15-69 years and 28% for patients aged 70 years and over (logrank chi(2) = 43.7, P < 0.00001). The younger and older groups treated with radical intent had complete response rates of 97% and 74%, respectively (logrank chi(2) = 17.91, P < 0.00001) and relapse rates at 5 years of 27% and 56%, respectively (logrank chi(2) = 4.86, P = 0.0275). The main reason for the poorer prognosis of patients aged 70 years and over was the increasing difficulty of chemotherapy delivery associated with advancing age.

Male breast cancer: pathological and immunohistochemical features.

BACKGROUND: male breast cancer is uncommon and studies regarding the potential clinical relevance of the histopathological and immunohistochemical features are infrequently reported. MATERIALS AND METHODS: We investigated the biological characteristics of forty-one male patients with invasive breast cancer by assessing histopathological and multiple immunohistochemical features. RESULTS: The majority were no special type (ductal) (37/41), lobular cancer was not seen. 73% were histological grade 3, 93% were positive for oestrogen receptor and 73% for progesterone receptor. The proportion of cancers positive for c-ebB-2 (45%), EGFR (20%), p53 (58%), MiB1 (40%), NCRC11 (78%), were similar to reports for female breast cancer. Nonsignificant associations between poor survival outcome and grade 3 tumours, and positive tissue staining for MiB1 and p53 were seen. CONCLUSION: While there ar similarities in the biological features of breast cancer in males and females, some differences were identified. Male breast cancer is more likely to be grade 3 tumours and hormone receptor positive.

A comparison outcome of male breast cancer with female breast cancer.

BACKGROUND: It is unclear whether breast cancer has a similar prognosis in males and females. METHODS: A 20-year retrospective study of all male breast cancer patients in our region was undertaken. We compared this series with a group of females matched for the major prognostic factors and an unmatched series of female patients treated over the same period. RESULTS: Forty-one patients with invasive cancer and 2 with ductal carcinoma in situ were identified. One invasive cancer was treated with radiotherapy, 40 had surgery. Local recurrence occurred in 23% and axillary recurrence in 40% of cases. Male and female patients (n = 123) matched for the major prognostic factors showed a similar outcome for disease-free interval (P = 0.90) and survival (P = 0.27). However, both the above groups had a significantly worse outcome than the unmatched series of female patients with breast cancer. CONCLUSIONS: When prognostic factors are allowed for, male and female breast cancer patients have a similar outcome. This suggests that such features should be taken into account when determining management for males with breast cancer just as they are in females.

Non-Hodgkin's lymphoma of the thyroid: a retrospective review of all patients diagnosed in Nottinghamshire from 1973 to 1992.

The pathological and clinical features were reviewed of all primary non-Hodgkin's lymphomas (NHL) of the thyroid gland diagnosed between 1973 and 1992 in the population (1.1 million) served by the Nottingham and North Nottinghamshire Health Authorities. Of the 43 patients with histologically proven NHL, three had low grade mucosa associated lymphoid tissue (MALT) lymphomas (Stage IEA, 2; Stage IIEA, 1), 35 had intermediate or high grade lymphomas, Stage IEA or IIEA (intermediate MALT, 2; high grade MALT, 14; B-cell diffuse centroblastic, 17; anaplastic large cell (Ki-1) of null cell type, 1; high grade unclassifiable, 1), and one had unclassifiable NHL Stage IIEA. One patient had Stage IIIEA disease (high grade MALT) and three had stage IVA disease (high grade MALT, 2; B-cell diffuse centroblastic, 1). The median age was 68 years (range 45-86) with a female: male ratio of 6:1. For the 35 patients with intermediate or high grade thyroid NHL (Stages IEA and IIEA) the 5- and 10-year cause specific survival was 60%. The 21 patients treated between 1985 and 1992 initially with chemotherapy (except stage IEA (< 5 cm diameter) had a 5-year cause specific survival of 69% (95% CI 48-90) compared with 46% (95% CI 19-73) for the 14 patients treated between 1973 and 1984 with initial radiotherapy (Chi 2 = 1.62). The survival of those patients with intermediate or high grade MALT lymphomas was not significantly greater than of those patients with B-cell diffuse centroblastic NHL.

Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine.

Mastocytosis is a disorder characterised by abnormal mast cell proliferation. The diverse spectrum of clinical presentations is dependent on the tissues and organs involved. We report a rare case of aggressive systemic mastocytosis presenting in utero with diffuse cutaneous involvement, and haematological abnormalities. There is little published evidence to guide treatment.

The many faces of secondary syphilis: a re-emergence of an old disease.

There has been a recent dramatic re-emergence of syphilis in the UK. This article describes the varied clinical manifestations of secondary syphilis and describes the diagnosis and current guidelines for treatment, especially in association with human immunodeficiency virus infection.

Two squamous cell carcinomas arising in a linear epidermal naevus in a 28-year-old female.

The development of a squamous cell carcinoma (SCC) in a linear epidermal naevus is a rare event previously reported in long-standing and often extensive lesions of middle-aged to elderly patients. To the best of our knowledge, this case appears to be only the second report of an SCC arising in a linear epidermal naevus in a young patient and the first report of two SCCs developing in a small epidermal naevus, over a short time, in a young patient with no other risk factors.

Findings at necropsy in the heart of a patient with anticardiolipin syndrome.

A 40 year old man with a history of myocardial infarction and hypertension presented with transient cerebral ischaemic attacks, aortic regurgitation, a raised erythrocyte sedimentation rate, and thrombocytopenia. The anticardiolipin syndrome was diagnosed and he was treated with prednisolone and warfarin. He died two years later after the development of acute heart failure. At necropsy his heart showed widespread arteriolar thrombosis without vasculitis, recanalised large vessel occlusion, and a "post-inflammatory" valvulitis of the aortic valve.

Antiphospholipid antibody syndrome presenting with complex partial seizures and transient ischaemic attacks due to widespread small cerebral arterial thrombosis.

The neuropathological findings in a young man with the antiphospholipid antibody syndrome presenting with complex partial seizures and transient ischaemic attacks are described. Whilst some of his symptoms could be attributed to large vessel thrombosis, the dominant pathological process was widespread small cerebral arterial thrombosis causing extensive microinfarcts within the cerebral cortex. These findings help to explain the neurological manifestations of the antiphospholipid antibody syndrome.

A case of bilateral trench foot.

A case of severe bilateral trench foot is presented in a patient who lived rough for 3 weeks without removing his boots. Non-operative management yielded no clinical improvement and bilateral below-knee amputation was necessary. Histology revealed subcutaneous and muscle necrosis with secondary arterial thrombosis.

Nonsteroidal antiinflammatory drug-induced colonic stricture. An unusual cause of large bowel obstruction and perforation.

Nonsteroidal antiinflammatory drugs (NSAIDs) are widely prescribed drugs that can result in gastroduodenal ulceration. Adverse effects upon the small and large intestine are now more often recognized. Small intestinal obstruction secondary to both mucosal diaphragms and broad-based stenoses is reported, but colonic strictures appear to be less common. A case of NSAID-induced colonic stricture leading to large bowel obstruction and secondary cecal perforation (previously unreported) is presented. Twelve additional cases of NSAID-induced colonic stricture are reviewed from the literature and its possible pathogenesis is discussed.