Foreign body reaction to polymethylsiloxane gel (Bioplastique) after vocal fold augmentation.

STATEMENT OF PROBLEM: The consequences of vocal fold paralysis include voice change, airway problems and difficulty swallowing. Medialisation procedures using injected material have been used for many decades, with varying outcomes, mainly secondary to lifespan, tissue reaction or migration. Newer materials have recently become clinically available which are easier to manage and supposedly less likely to elicit foreign body reaction. METHOD OF STUDY: Case report. RESULTS: We report a case of foreign body reaction and possible migration of polymethylsiloxane gel (Bioplastique), one such material, after vocal fold injection. To our knowledge, this is the second such case described. CONCLUSIONS: This case highlights the fact that the risk of foreign body reaction and migration is still present for this material, albeit low. We also highlight the fact that, although this material can cause foreign body reactions and may possibly migrate, it is removable by microlaryngoscopy via the microflap technique, with vocal improvement.

Anaplastic large cell lymphoma with ALK expression and presence of the t(2;5) translocation in a 5-month-old infant.

Anaplastic large cell lymphoma (ALCL) is a well-recognized subtype of non-Hodgkin lymphoma in childhood. Several series report experience with the diagnosis and management of pediatric ALCL, the average age at diagnosis being 8 to 16 years, with a reported range of 1 to 15 years. We present a case of ALCL affecting a 5-month-old infant in whom the diagnosis was confirmed by the nuclear and cytoplasmic immunohistochemical expression of ALK1, in addition to the presence of classical t(2;5)(p23;q35) translocation detected using reverse transcriptase-polymerase chain reaction. This is the youngest case of ALCL thus far reported and hence expands the spectrum of infantile lymphoproliferative disorders.

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.

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.

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.

Massive cardiac chondroma presenting with heart failure and superior vena cava obstruction in a teenage boy.

Histologically benign soft-tissue chondromas have been reported at many anatomical sites but are an uncommon cause of soft tissue mass lesions in childhood, accounting for less than 1% of cases. The most frequent sites for extraosseous soft-tissue chondromas are the hands and feet. For the extremely rare visceral chondromas, the site can be lung, where they may represent a component of Carney's syndrome of extra-adrenal paraganglioma, pulmonary chondroma, and epithelioid leiomyosarcoma of the gastrointestinal tract. Primary cardiac chondromas are exceptionally rare in patients of any age although cardiac chondrosarcoma, both primary and metastatic, is well reported. We present a case of a teenage boy with a fatal cardiac chondroma

Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases.

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth--3 years versus median 1 week, range birth--10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z =1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.