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Biomedical subjects

A D Ramsay

Publications and source records attributed to A D Ramsay.

At least 19 recordsLinked to original sources

Epstein-Barr virus-associated lymphoproliferative disorder presenting as apparently isolated gastrointestinal lesions in childhood.

We present an unusual case of post-transplant lymphoproliferative disorder (PTLD) presenting as apparently isolated gastrointestinal lesions in a pediatric renal transplant recipient. The multiple bowel lesions were related to Epstein-Barr virus and demonstrated the appearance of a monomorphic PTLD that was morphologically indistinguishable from diffuse large B-cell lymphoma. The patient responded to therapy with targeted anti-CD20 immunotherapy. PTLD may manifest as apparently isolated gastrointestinal tract lesions in childhood.

Antibodies, Monoclonal↗

Histopathological features of open lung biopsies in children treated with extracorporeal membrane oxygenation (ECMO).

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has become an established treatment for severe respiratory distress in a range of pediatric conditions. This study describes the histopathological features in a series of 22 children receiving ECMO therapy in whom open lung biopsy was carried out. AIMS: To describe the histopathological features of open lung biopsies in children receiving ECMO therapy. STUDY DESIGN: Retrospective review of clinical material. SUBJECTS: Children receiving ECMO therapy in whom open lung biopsy was carried out. RESULTS: In those investigated in infancy, open lung biopsy allowed a definite diagnosis to be made of the underlying condition in more than 90% of cases. In older children, the histopathological changes were more non-specific and, although providing useful clinical information, a definitive diagnosis could often not be made. In about a quarter of cases, there are additional pathological features, which may be related to ECMO treatment, such as significant intra-alveolar haemorrhage, but ECMO does not in itself impair the diagnostic usefulness of open lung biopsy in these selected patients. CONCLUSION: Open lung biopsy provides clinically useful information in infants receiving ECMO therapy. The histopathological changes may be complex and represent both the effects of ECMO and progression of the underlying disease.

Age Factors↗

Juvenile angiofibroma of the maxillary sinus.

Primary extra-nasopharyngeal sites of angiofibromas are extremely unusual. We describe a rare case of extra-nasopharyngeal angiofibroma in a one year old child arising from the left maxilla and indirectly involving the lacrimal system. The initial presentation was of a swelling in the region of the left medial canthus. Only four cases of extra-nasopharyngeal angiofibromas in children below the age of two have been described. We review the literature on what is known about extra-nasopharyngeal angiofibromas.

Angiofibroma↗

Isolated EBV lymphoproliferative disease in a child with Wiskott-Aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy.

Patients with primary immunodeficiencies such as the Wiskott-Aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. Biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.

Adolescent↗

Fatty degeneration in a Wilms' tumour after chemotherapy.

We report a case of extensive fatty change in a Wilms' tumour after chemotherapy demonstrated on CT associated with an increase in tumour volume, in a 10-month-old girl with Beckwith-Wiedemann syndrome. Changes in tumour characteristics after chemotherapy on imaging usually reflect necrosis, haemorrhage and calcification. Assessment of response to therapy is dependent on a documented reduction in tumour volume. In this case, CT showed an increase in tumour size with development of an extensive fatty component following treatment. Subsequent histological examination on the nephrectomy specimen confirmed an extensive fatty component with no evidence of residual blastema. The development of such an extensive fatty component is very unusual. In this case such fatty change was an indicator of tumour sensitivity and response to treatment.

Antineoplastic Combined Chemotherapy Protocols↗

A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy.

OBJECTIVE: The purpose of this study was to review the features of 8 cases of melanotic neuroectodermal tumor of infancy (MNTI) of the jaws with respect to the expression of NB84, CD99, PGP 9.5, specific cytokeratins, and Ki-67, markers not previously reported in this entity. STUDY DESIGN: A clinicopathologic and immunohistochemical analysis of MNTIs in 8 children was undertaken. RESULTS: Patients were aged 2(1/2) months to 14 months. Seven were males. Seven lesions affected the maxilla. Microscopically, collections of larger, melanocyte-like cells were admixed with smaller, neuroblast-like cells. All MNTIs contained melanin; although most showed cellular atypia, mitoses were infrequent (<2 per 10 high-power fields). However, in one lesion in which the melanocyte-like cells appeared less differentiated, 7 mitoses per 10 high-power fields were counted. The larger cells expressed cytokeratins 7 (4/8), 8 (8/8), 18 (6/8), and 19 (3/8); PGP 9.5; neuron-specific enolase (6/8); S100; HMB45; and chromogranin A (2/8). The small cells expressed CD56 (7/8), neuron-specific enolase (7/8), synaptophysin (3/8), PGP 9.5 (3/8), and chromogranin A (2/8). No MNTIs expressed NB84. The most mitotically active tumor was the only one to show membrane expression of CD99 (by both cell populations), have a detectable Ki-67-positive fraction (25% in both the large- and small-cell components), behave aggressively, and require bilateral maxillectomy. All other MNTIs responded to local excision, and none metastasized. CONCLUSIONS: Most MNTIs are benign and respond to conservative excision. Histology is an unreliable means of predicting clinical behavior, but this study has identified some morphologic and phenotypic features that may indicate a more aggressive lesion.

12E7 Antigen↗

Laryngeal paraganglioma in a five-year-old child--the youngest case ever recorded.

A paraganglioma is a neuroendocrine neoplasm that originates from the paraganglion cells of the parasympathetic system. The average age of presentation is in the fifth decade. We report a case of laryngeal paraganglioma in a five-year-old child, the youngest case ever recorded. The features of paraganglioma which differentiate it from other tumours are also discussed.

Age Factors↗

Errors in histopathology reporting: detection and avoidance.

The histopathological diagnosis is the bedrock of modern oncology, and plays a major role in the treatment of many other types of disease. Errors in these reports can critically affect patient care and may become the subject of media concern. This article considers how audit in histopathology can provide information about errors and inconsistencies in the diagnosis of surgical specimens. The use of audit to generate information about the background level of errors in pathology reports is reviewed, along with findings about the nature of these errors and the types of specimens more commonly affected. Generic audit strategies that can be used to minimize the risk of errors in reports are discussed, together with the use of audit to evaluate diagnostic criteria and pathological scoring or grading systems. The role of audit in determining the informational content of reports is included, and there is consideration of the relationship between sample size and error rates. The limited extent to which audit can be used to assess the performance of individual pathologists is also covered.

Diagnosis↗

Is the binding of the lectin Helix pomatia agglutinin (HPA) of prognostic relevance in tumours of the upper aerodigestive tract?

The lectin Helix pomatia agglutinin (HPA) has been used as a prognostic indicator in a number of clinical studies including those of breast, colorectal and gastric cancer. Binding of HPA to tissue sections was associated with a bad prognosis indicating that the carbohydrate residue recognized by this lectin is linked to metastasis. In order to investigate whether HPA binding is also of prognostic relevance in squamous cell carcinomas of the upper aerodigestive tract, 53 tumours of this region were stained with HPA. Almost all tumours (95%) bound HPA to various degrees and hence HPA binding is of no prognostic relevance in this group of tumours. These findings indicate a fundamental difference in the role of carbohydrate residues in metastasis between squamous cell carcinoma (as in our study) and in tumours derived from glandular tissues such as breast, colon and stomach.

Adenocarcinoma↗

Primary neuroblastoma of the facial nerve presenting as a recurrent facial paralysis.

A case is described of a primary neuroblastoma of the facial nerve in a 13-year-old boy presenting with a recurrent facial paralysis. This tumour was excised preserving the nerve and followed with post-operative radiotherapy. The pathology of the tumour is described and facial nerve tumours briefly discussed as a cause of facial palsy. There have been no other cases of a primary neuroblastoma of the facial nerve arising at this site reported in the last 20 years.

Adolescent↗

Idiopathic tracheal stenoses.

The case histories are presented of three patients with circumferential subglottic stenoses who presented, over a six-month period, to a teaching hospital's Otolaryngology department. No recognisable cause for their subglottic stenoses was found. Traumatic, iatrogenic, infectious and specific inflammatory processes were excluded. The histopathological evidence suggested a chronic inflammatory process. All patients were treated by resecting the stenosis with a carbon dioxide laser. One patient required an emergency tracheostomy for airway obstruction. A review of the published literature on this rare condition is given.

Adult↗

Primary cervical lymphoma: the role of cervical cytology.

Two cases of primary malignant lymphoma of the uterine cervix are reported. Both were confirmed by histology as high grade B cell lymphomas. In one case, the diagnosis was made on a second colposcopic biopsy after an initial cervical smear and colposcopic biopsy were negative. In the second case, dyskaryotic cells of uncertain type were identified in a cervical smear taken at colposcopy performed as part of follow up for previous cervical intraepithelial neoplasia (CIN)I. The cytologic features and differential diagnosis of this rare cervical neoplasm are discussed, with emphasis on the role of the Papanicolaou smear in the initial diagnosis of this tumour.

Adult↗

Aluminium and injection site reactions.

AIMS: To alert pathologists to the spectrum of histological appearances that may be seen in injection site reactions related to aluminium. METHODS: Four cases of injection site reaction were examined microscopically using routine staining with haematoxylin and eosin, electron microscopy and by electron probe microanalysis. RESULTS: As in previous reports, all four cases included collections of histiocytes which contained faint granular brownish refractile material within their cytoplasm; ultrastructural examination showed this to be aluminium. Two cases showed a prominent inflammatory reaction with numerous lymphoid follicles and a notable eosinophilic infiltrate. Two cases showed unusual features not described previously. In one, there was a sclerosing lipogranuloma-like reaction with unlined cystic spaces containing crystalline material. The other case presented as a large symptomatic subcutaneous swelling which microscopically showed diffuse and wide-spread involvement of the subcutis by a lymphoid infiltrate with prominent lymphoid follicles. CONCLUSIONS: This report highlights the changes encountered in aluminium injection site reactions and emphasises that the lesions have a wider range of histological appearances than described previously.

Adult↗

Localization of Epstein-Barr virus in Castleman's disease by in situ hybridization and immunohistochemistry.

Paraffin wax sections of lymph node biopsies from a total of thirteen patients with the morphologic and clinical features of Castleman's disease were analyzed for the presence of the Epstein-Barr virus (EBV) by in situ hybridization for the noncoding EBV early RNAs (EBERs) and by immunohistochemistry for the EBV-encoded latent membrane protein-1 (LMP-1). Of twelve cases of localized Castleman's disease EBER-positive cells were identified in five, and in these cases were only rarely found and were always confined to the interfollicular regions. LMP-1 was not detected in any of these cases, either alone or after dual staining for EBERs and LMP-1. (A similar pattern of EBER expression is seen in nonneoplastic lymphoid tissue from EBV-positive individuals.) No EBER-positive or LMP-1 positive cells were identified in a single case of multicentric Castleman's disease. In two additional patients initially diagnosed with Castleman's disease of localized plasma cell type, repeat biopsy showed Hodgkin's disease. In both cases Reed-Sternberg cells and their variants were identified in the original biopsy on which the diagnosis of Castleman's disease was made. In one of these cases these cells showed expression of EBERs and LMP-1, indicating latent infection with EBV. The results suggest that EBV is not generally associated with Castleman's disease. Further analysis of a series of cases of multicentric Castleman's disease is indicated.

Adult↗