Nasopharyngeal chondrolipoma.

In this case report, we describe the presentation and treatment of a patient with nasopharyngeal chondrolipoma. Lipomas are common soft tissue tumours, although their incidence in the nasopharynx is very low. A rarer variant of lipoma, chondrolipomas are benign mesenchymal tumours. They are formed by the proliferation of mature adipocytes and contain different amounts of mature cartilaginous tissue; Weiss "Enzinger and Weiss's soft tissue tumours", 4th ed: Mosby, St Louis; 2001 This represents the second reported case of a nasopharyngeal chondrolipoma. An endonasal approach to excision has not been previously described.

Hypoxia-inducible factor-1alpha expression in the gastric carcinogenesis sequence and its prognostic role in gastric and gastro-oesophageal adenocarcinomas.

Hypoxia-inducible factor-1 (HIF-1)alpha expression was studied in the gastric carcinogenesis sequence and as a prognostic factor in surgically resected gastric and gastro-oesophageal junction tumours. Protein expression was examined using immunohistochemistry on formalin-fixed biopsies of normal mucosa (n=20), Helicobacter pylori associated gastritis (n=24), intestinal metaplasia (n=24), dysplasia (n=12) and intestinal (n=19) and diffuse (n=21) adenocarcinoma. The relationship between HIF-1alpha expression and prognosis was assessed in resection specimens from 177 patients with gastric and gastro-oesophageal junction adenocarcinoma. Hypoxia-inducible factor-1alpha expression was not observed in normal gastric mucosa but increased in density (P<0.01) and intensity (P<0.01) with progression from H. pylori-associated gastritis, intestinal metaplasia, dysplasia to adenocarcinoma. The pattern of staining in the resection specimens was focally positive in 49 (28%) and at the invasive tumour edge in 41 (23%). Invasive edge expression was associated with lymph node metastases (P=0.034), advanced TNM stage (P=0.001) and was an adverse prognostic factor for cancer-specific survival (P=0.019). In univariate analysis and in comparison with tumours not expressing HIF-1alpha, invasive edge staining was associated with a hazard ratio of 1.6 (95% CI 1.0-2.5) and focally positive staining a hazard ratio of 0.7 (95% CI 0.5-1.2). Hypoxia-inducible factor-1alpha lost prognostic significance in multivariate analysis. The results suggest HIF-1alpha is involved in gastric carcinogenesis and disease progression, but is only a weak prognostic factor for survival.

Histiocytic necrotising lymphadenitis (Kikuchi's disease): a rare cause of cervical lymphadenopathy.

Both Head and Neck Surgeons and General Surgeons are frequently referred patients with cervical lymphadenopathy. An uncommon but important cause is histiocytic necrotising lymphadenitis. This is a benign self-limiting disease that has been confused with malignant lymphomas. Some patients may also experience distressing and debilitating symptoms which can last for months. We describe four cases to illustrate the varied clinical presentation of this disease and present new signs seen in association with it. A remarkable therapeutic response to a short course of oral corticosteroids was observed in one case.

Pulmonary (99m)Tc-DTPA aerosol clearance and survival in usual interstitial pneumonia (UIP).

BACKGROUND: Clearance of inhaled technetium 99m-labelled diethylenetriamine penta-acetic acid ((99m)Tc-DTPA) from the lungs is a potential indicator of disease progression in patients with idiopathic pulmonary fibrosis (IPF). METHODS: We prospectively analysed the usefulness of this technique for predicting survival in 106 non-smoking patients with usual interstitial pneumonia (UIP) pattern IPF diagnosed by high resolution CT (HRCT) scanning or histological examination (M/F 65/41, mean (SD) age 61 (11) years). DTPA clearance was analysed according to both mono-exponential and bi-exponential models. Half times for the fast (t(0.5)F) and slow (t(0.5)S) components of clearance, the percentage contribution of the fast component (fF), and half time for mono-exponential approximation to the early part of the clearance curve (t(0.5)) were calculated. RESULTS: The patients had substantially faster t(0.5) (mean 23.9 (9.6) minutes) than normal values (>45 minutes). Thirty seven patients (35%) died during follow up (median 15 months). Univariate Cox regression analysis identified significant predictors of survival as age, forced expiratory volume in 1 second (FEV(1)), forced vital capacity (FVC), total lung capacity (TLC), % predicted TLC, carbon monoxide transfer factor (TLCO), % predicted TLCO, arterial oxygen tension (PaO(2)), oxygen saturation, t(0.5)F, and HRCT fibrosis score. Multiple stepwise Cox regression analysis identified t(0.5)F (p=0.03, hazard ratio 0.747, 95% CI 0.578 to 0.964), % predicted TLC (p=0.02, hazard ratio 0.976, 95% CI 0.956 to 0.995), % predicted TLCO (p=0.003, hazard ratio 0.960, 95% CI 0.935 to 0.986), and age (p=0.003, hazard ratio 1.062, 95% CI 1.021 to 1.104) as independent predictors of survival. CONCLUSION: These data suggest that (99m)Tc-DTPA clearance t(0.5)F measurement may predict survival in patients with UIP pattern IPF.

Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation.

Of patients awaiting lung transplantation, the death rates are highest in those with idiopathic pulmonary fibrosis (IPF), suggesting that many IPF patients are referred late for transplantation. Therefore this study was undertaken to evaluate baseline pulmonary function test (PFT) and high-resolution computed tomography (HRCT) fibrosis scores, and the relationship to survival in IPF patients younger than 65 yr of age. A total of 115 patients with usual interstitial pneumonia (UIP) were studied. At presentation to a tertiary referral center, PFT and HRCT data were collected and analyzed for prognostic significance: the primary outcome measure was patient death. Based on the length of the waiting list for transplantation, prediction of 2-yr survival was examined. DL(CO) percent predicted and HRCT-fibrosis score were found to be independent predictors of survival and in combination gave the best prognostic prediction. The optimal points on the receiver operating characteristic (ROC) curves for discriminating between survivors and nonsurvivors corresponded to 39% DL(CO) percent predicted, and to a HRCT-fibrosis score of 2.25. The combination of these parameters yielded an optimal point with a specificity and a sensitivity of 84% and 82%, respectively. A model based on a combination of DL(CO) percent predicted and HRCT-fibrosis score may optimize the timing of referral for transplantation.

Bone histomorphometry in adult patients with cystic fibrosis.

STUDY OBJECTIVE: Low bone mineral density is a common complication of cystic fibrosis (CF), and recent studies have implicated vitamin D insufficiency as a significant etiologic factor. The aim of this study was to establish whether there was bone biopsy evidence of vitamin D deficiency osteomalacia in patients with CF and to document the general histomorphometric characteristics of CF bone. PATIENTS AND METHODS: A retrospective descriptive and histomorphometric study of postmortem L2/L3 vertebral bone biopsy specimens was undertaken on tissue from 11 posttransplant CF patients and 4 nontransplanted CF patients. Control data were derived from postmortem bone specimens from 15 young adults. RESULTS: Bone from all CF patients was characterized by severe osteopenia in both trabecular and cortical bone. At the cellular level, there was decreased osteoblastic and increased osteoclastic activity. The reduction in osteoblastic activity was due to both a decrease in osteoblast number and a decrease in the biosynthetic potential of osteoblasts. The osteoclastic changes were due to an increase in the number of osteoclasts. The increase in osteoclasis and the uncoupling of osteoblastic and osteoclastic activity resulted in an increase in resorptive surfaces. Although there were few significant differences between the transplanted and nontransplanted CF groups, both cortical and trabecular bone mass tended to be lower after transplantation. None of the CF undecalcified biopsy specimens showed osteoid parameters characteristic of vitamin D deficiency osteomalacia. CONCLUSIONS: CF patients have an unusual and complex pattern of cellular changes within bone that are not typical of vitamin D deficiency osteomalacia.

Intracardiac thrombus in Behçet's disease: a systematic review.

BACKGROUND: Intracardiac thrombus formation is a rare but serious complication of Behçet's disease. We aimed to review the clinical and pathologic correlates of cardiac thrombus formation in the context of Behçet's disease. METHODS AND RESULTS: A comprehensive search of the medical literature was conducted using MEDLINE including bibliographies of all selected articles. Although the disease has a unique geographic distribution, being most common in the population of the ancient Silk Route, cases complicated by intracardiac thrombus have mostly originated from the Mediterranean basin and the Middle East. Young men appear to be most at risk, with the right heart the most frequent site of involvement. The first symptoms and signs of the disease frequently precede systemic organ manifestations. In those cases in which intracardiac thrombus occurs, it is apparent in more than half of cases on first recognition of the disease. CONCLUSION: A diagnosis of Behçet's disease should be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical features of the condition. This is particularly applicable if the patient is a young man from the Mediterranean basin or the Middle East.

Endobronchial metastasis from osteosarcoma of bone: treatment with intraluminal radiotherapy.

Lung parenchymal metastases are common manifestations in patients with osteosarcoma; however, spread to the major airway itself is extremely rare. We present a young man who had been previously treated with surgical resection following preoperative chemotherapy and immediate postsurgical adjuvant chemotherapy for proximal tibial osteosarcoma. He developed metastasis to the major airways. The patient was treated with intraluminal radiotherapy (ILT) for the endobronchial metastasis. This is the first report of an endobronchial osteosarcoma that was treated with ILT with a complete endoscopic response. ILT provided excellent palliation in this particular case.

Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation.

We describe two patients with histologically proven pulmonary Langerhans' cell histiocytosis in whom radiologic improvement occurred following smoking cessation. The patients had 23- and 25-pack-year smoking histories, respectively. High-resolution CT revealed multiple small nodules, located predominantly in the upper and middle lung fields. There was a close temporal relationship between smoking cessation and radiologic improvement.

Differentiation of desquamative interstitial pneumonia (DIP) from pulmonary adenocarcinoma by immunocytochemistry.

AIM: After a misdiagnosis of pulmonary adenocarcinoma as desquamative interstitial pneumonia (DIP), we investigated whether immunohistochemical markers could differentiate these conditions. METHODS AND RESULTS: Three cases of DIP and one pulmonary adenocarcinoma masquerading as DIP were studied by light and electron microscopy. All cases were mucin-negative. The cases of DIP were CD68 positive but cytokeratin-negative. The adenocarcinoma was cytokeratin-positive (AE1/3 and CAM5.2), as well as showing some CD68-positive cells. Markers for carcinoma (CEA, Ber-EP4, and Leu M1) were negative in all cases. Ultrastructurally the adenocarcinoma appeared to be derived from Type II pneumocytes. CONCLUSION: Before a diagnosis of DIP is made, cytokeratin markers should be used.

Nodular erythema elevatum diutinum mimicking cutaneous neoplasms.

AIMS: We describe the cutaneous pseudoneoplastic lesions in two patients with nodular erythema elevatum diutinum, a rare chronic disorder in which polymorph nuclear fragmentation (leukocytoclasis) is present within dermal nodules showing spindle cells and fibrosis. In both cases diagnostic difficulty was encountered clinically and pathologically and various benign and malignant neoplasms were considered in the differential diagnosis. METHODS AND RESULTS: Immunohistochemically the spindle cells were negative for CAM5.2, AE1/3, S100 protein and desmin (D33). They were positive for vimentin and focally positive for CD34 and alpha-smooth muscle actin. Some of the spindle cells were positive for Mac 387 and KP1(CD68). By electron microscopy, the lesions were shown to consist of fibroblasts/myofibroblasts and fusiform macrophages. CONCLUSIONS: Increased awareness of the features described will help to avoid misdiagnosis as a neoplastic process.

Malignant melanoma showing smooth muscle differentiation.

A unique case of a metastatic non-desmoplastic sarcomatoid malignant melanoma in an axillary lymph node showing smooth muscle differentiation in a 54 year old woman is described. The tumour cells exhibited alpha-smooth muscle actin, HHF-35 and desmin positivity but were negative for S100 protein and HMB-45. Ultrastructural examination revealed smooth muscle phenotype and there was no evidence of myofibroblastic differentiation, a feature described previously in desmoplastic melanomas.

Inflammatory pseudotumour of lymph nodes.

AIM: To describe the clinical, histological and immunohistochemical features in four cases of an uncommon benign lymph node lesion which may mimic a neoplastic process. METHODS: Four cases of inflammatory pseudotumour of lymph nodes were studied using conventional staining (haematoxylin and eosin, PAS, Gordon and Sweets reticulin stain, and the Ziehl-Neelsen stain) and with immunohistochemical techniques using a variety of antibodies (CD3, L26, CD15, CD21, CD30, KP1, MAC 387, vimentin, alpha SMA, HHF-35, D33, CD34, and S100). RESULTS: The lesion comprises a proliferation of spindle cells expanding the connective tissue framework of lymph nodes and is associated with a plasma cell and small lymphocyte infiltrate. There are variable numbers of macrophages, neutrophils and eosinophils, and varying degrees of fibrosis. Vascular changes are common but vary in degree and type. CONCLUSIONS: Inflammatory pseudotumour of lymph nodes is an uncommon benign reaction pattern which may be misdiagnosed as a neoplastic or even a malignant process. Increased awareness of its histological features should help prevent such misdiagnoses.

An immunophenotypic survey of malignant melanomas.

An immunohistochemical study of 106 malignant melanoma specimens from 59 patients, using formalin-fixed, paraffin-embedded material, is reported. Negativity for HMB-45 was seen in 11% of specimens. The rate of positivity with CAM 5.2 was 7%. One specimen showed alpha-smooth muscle actin (alpha SMA) positivity. For 11 of the 12 cases in which anomalous immunophenotypes were seen, multiple specimens were available; nine of these showed evidence of an alteration in the immunophenotype between specimens. Comparing the primary tumours with local recurrences and metastases, there was, variously, loss of HMB-45, S-100 protein and NKI/C3 positivity, and acquisition of CAM 5.2 and alpha SMA positivity. In some cases, the change of immunophenotype appeared to occur in a single step. However, one case with six consecutive specimens showed evidence of progressive loss of HMB-45, S-100 protein and NKI/C3 with concomitant gain of CAM 5.2 staining. The implications for the use of immunophenotyping in diagnostic practice are discussed.

Audit of bone marrow trephines.

AIMS: To establish criteria of adequacy for bone marrow trephine biopsy specimens and to audit the quality of trephines performed at the Christie Hospital, Manchester. METHODS: Trephines (n = 767) performed over 12 months were reviewed. Their lengths, and the lengths of their constituent parts (soft tissue, cortex, crushed marrow and interpretable marrow) were measured. The mean performance of each operator was calculated. Criteria of adequacy were established by a review of the published findings and an analysis of the relation between trephine length and the rate of infiltration by tumour. RESULTS: Before processing, the average trephine was 1.59 cm long. Trephines shrunk by 25% during processing. In histological sections the average length was 1.15 cm, consisting of 0.09 cm of soft tissue, 0.04 cm of cortex, 0.26 cm of disrupted marrow and 0.74 cm of interpretable marrow. A large number of operators were taking trephine biopsy specimens and their performance varied considerably. Review of the published findings suggested that the minimum adequate length is in the range 1.5 cm to 2.0 cm. The analysis of the relation between length of trephine and the rate of positivity for neoplasia yielded a minimum adequate length of 1.2 cm in section (1.6 cm before processing). Fifty eight per cent of the trephines were inadequate by this criterion. There was a tendency for the Jamshidi needle to produce a longer trephine than the Islam needle. CONCLUSION: According to objective criteria, at the Christie Hospital, many operators are producing a high proportion of inadequate bone marrow trephines.