Pittsburgh Classification Research Articles

Treatment outcome and prognostic factors of external auditory canal squamous cell carcinoma: A retrospective study in a tertiary center.

Squamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare malignancy with various treatment strategies and outcomes. The purpose of this study was to evaluate the clinical characteristics and survival outcomes and identify prognostic factors in patients with SCC of EAC. Twenty-one patients with SCC of EAC treated in a single tertiary center between 2009 and 2021 were retrospectively reviewed and analyzed. The modified Pittsburgh classification system was applied for staging. Factors associated with survival were identified by univariate survival analysis. The mean age at diagnosis was 61 years (range: 41-79 years). Early-stage (T1 + T2) accounts for 38.1% of the series and advanced-stage (T3 + T4) accounts for 61.9%. Eighteen (85.7%) patients underwent primary surgery with curative intent. The 5-year overall survival rate of the 21 patients was 67.4%. Tumor invasion to the otic capsule, eustachian tube, sigmoid sinus, and dura were associated with poor prognosis in univariate analysis (p = .046; .008; .027; and .08, respectively). Factors predictive of less favorable survival include the history of COM, tumor invasion to the otic capsule, eustachian tube, sigmoid sinus, and dura. It is important to make a precise and systemic preoperative evaluation of disease extent. 4.

Unraveling a Major Burden of Orofacial Clefts Analyses: Classification of Cleft Palate Fistulas by Cleft Surgeons.

The objective of this study was to investigate how cleft surgeons classify palatal fistulas. We focused on three different anatomical locations (ie, hard palate, soft palate, junction hard/soft palate) to analyze agreement/disagreement at various anatomical locations. Cross-sectional survey study. Participants in an international webinar that focused on palatal fistula treatment were included. Participants were presented with a survey pre- and post-webinar. Frequency of used classification systems for classifying oronasal fistulas and the inter-rater reliability of the Pittsburgh classification system. A total of 141 participants completed the questionnaires prior to the webinar and 109 participants completed the survey after the webinar. In total, four classification systems were used (ie, Pittsburgh, Pakistan Comprehensive Fistula Classification [PCFC], anatomical and 'other'). The Pittsburgh classification was the most commonly used system in all cases. However, Pittsburgh inter-rater reliability was low (κ = 0.136 pre-webinar, and κ = 0.174 post-webinar). Surprisingly, a substantial shift was observed from the anatomical to Pittsburgh classification after the webinar, indicating increased awareness of the usability of the Pittsburgh classification system. This study demonstrates a large heterogeneity with regards to the classification of cleft palate fistulas. Interestingly, a shift was observed from the anatomical to Pittsburgh classification after the webinar. However, the inter-rater reliability for using the Pittsburgh classification was low. Classifying palatal fistulas in a homogenous fashion could enhance comparison of primary palate repair and could improve treatment of palatal fistulas.

Outcome of Nasal Layer Reinforcement With Autologous Dermis in Cleft Palate Repair on Postoperative Fistula Formation.

Palatal fistulae are challenging complications following cleft palate repair. The addition of acellular dermal matrix (ADM) to cleft palate repair has been shown to reduce fistula formation in previous studies. The use of autologous dermal graft has all the structural advantages of ADM, has less rejection and immunogenic potential, and is cost effective. A prospective study. Patients with Group II and III cleft palate (Nagpur Classification) without prior intervention for palatal repair in the Department of Plastic Surgery at PGIMER from January 2020 till June 2021. The addition of autologous dermal graft for palatoplasty. Outcome of the study was fistula development or exposure of dermal graft. Autologous dermal graft was harvested of average dimension of 8.73 cm2 (range 5.25-18 cm2) from groin region. Sixteen patients were included in the study. Among them, 2 patients (12.5%) developed postoperative fistula (Type III &V Pittsburgh Classification). Our study showed that the rates of postoperative fistula formation are comparable with prior literature using artificial dermal matrices.

Clinical Analysis of En Bloc Resection for Advanced Temporal Bone Squamous Cell Carcinoma.

Objective En bloc and margin-negative surgical resection seems to offer the best prognosis for patients with temporal bone squamous cell carcinoma (TB-SCC). In this study, we summarize the outcomes of surgical cases of advanced TB-SCC (T3-T4) that were managed in two institutions, with an accompanying description of the surgical procedure that was utilized: modified subtotal temporal bone resection (STBR), which involves the en bloc removal of the temporal bone including or transecting the otic capsule. Design This is a case series study with chart review. Setting The study was conducted at two academic tertiary care medical centers. Participants Chart information was collected for all patients who underwent surgical resection of advanced TB-SCC between July 1998 and February 2019. The resulting dataset contained 43 patients with advanced TB-SCC who underwent en bloc resection during the review period. Tumor staging followed the modified Pittsburgh classification. Disease-specific survival (DSS) rates were calculated according to the Kaplan-Meier method. Main Outcome Measure This study shows disease-specific 5-year DSS rate. Results The 5-year DSS rate of the cases who underwent en bloc resection was 79.7%. En bloc lateral temporal bone resection was employed in a total of 25 cases (DSS: 79.0%). En bloc modified STBR was utilized in 18 cases (DSS: 81.7%). Conclusion En bloc margin-negative resection is a reliable treatment strategy for advanced TB-SCC. Modified STBR can be a treatment option for TB-SCC without marked posterior extension.

A retrospective analysis of radiotherapy in the treatment of external auditory canal carcinoma.

External auditory canal carcinoma (EACC) is a rare disease. The current study aimed to evaluate the clinical outcomes of patients treated with external beam radiotherapy (EBRT) for EACC. The present study retrospectively reviewed 34 consecutive patients treated for EACC with EBRT between February 2001 and January 2019 at the University of Tokyo Hospital. Clinical staging was performed according to the modified Pittsburgh classification. Of all the included patients, seven patients were in the early stages (I or II) and 27 in the advanced stages (III or IV) of EACC. A total of 16 patients underwent EBRT and surgery (S+RT) pre- and/or postoperatively, while 18 patients underwent definitive radiotherapy (dRT). The median prescribed doses for the S+RT and dRT groups were 66 and 70 Gy, respectively. The median follow-up period for all patients was 22.4 months (range, 2-205 months). The 5-year overall survival rates of the S+RT and dRT groups were 66.7 and 45.1%, respectively. The progression-free survival rate at 5-year was 55.6% (95% confidence interval: 36.5-71.1%) for the entire cohort. A total of 14 patients experienced disease relapse after treatment, consisting of 11 locoregional recurrences and three distant metastases. The current study revealed the clinical outcomes of EBRT for EACC.

Evaluation of the modified Pittsburgh classification for predicting the disease-free survival outcome of squamous cell carcinoma of the external auditory canal.

BackgroundSquamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare disease, which is commonly classified with the modified Pittsburgh classification. Our aim was to evaluate the predictive performance of this classification in relation to disease‐free survival (DFS).MethodsWe examined retrospective data from a nationwide Dutch cohort study including patients with primary EAC SCC. These data were combined with individual patient data from the literature. Using the combined data, the predictive performances were calculated using the c‐index.ResultsA total of 381 patients were included, 294 for clinical and 281 for the pathological classification analyses. The c‐indices of the clinical and the pathological modified Pittsburgh classification predicting DFS were 0.725 (0.668‐0.782) and 0.729 (0.672‐0.786), respectively.ConclusionThe predictive performance of the modified Pittsburgh classification system as such appears to be acceptable to predict the DFS of EAC SCC. Other factors need to be added to a future model to improve the predicted performance.

Therapeutic effect of Nivolumab for advanced / recurrent temporal bone squamous cell carcinoma

ObjectiveThe immune checkpoint inhibitor Nivolumab was approved for the treatment of platinum-refractory head and neck squamous cell carcinoma (SCC), expanding the treatment options for recurrent or advanced head and neck SCC. However, since temporal bone squamous cell carcinoma (TB-SCC) is very rare cancer, the effectiveness of Nivolumab remains unclear. We investigated the effects of Nivolumab for TB-SCC. MethodChart information was collected for all patients who underwent the first administration of Nivolumab for recurrent or residual TB-SCC in our hospital between September 2017 and December 2019. Tumor staging followed the modified Pittsburgh classification. Changes in the tumor burden and survival outcome were examined. ResultsWe examined 9 patients with recurrent or residual TB-SCC who started administration of Nivolumab. In these cases, recurrent or residual SCC was observed after chemotherapy and/or chemoradiotherapy including platinum. The duration of Nivolumab was 2–54 weeks (median 20.0 weeks). The evaluation of the therapeutic effect according to the RECIST method showed partial response in 1 case, stable disease in 2 cases, progressive disease in 4 cases, and size unevaluated in 2 case. Although the number of cases was small, comparing with 5 cases without Nivolumab, these cases showed longer overall survival (1-year OS 33.3% vs 20.0%). ConclusionWe used Nivolumab as palliative chemotherapy in 9 patients with recurrent/residual TB-SCC, and we were able to obtain a certain therapeutic effect on TB-SCC as well as other head and neck SCC.

The Clinical Characteristics and Surgical Outcomes of Carcinoma of the External Auditory Canal: A Multicenter Study.

To analyze the clinical characteristics and evaluate the surgical outcomes of carcinoma of the external auditory canal (CEAC). Overall, 31 patients from four multicenter hospitals, who were diagnosed and surgically treated for CEAC in 2009-2014, were enrolled for this retrospective study. Medical records were reviewed to determine cancer stage according to the Pittsburgh classification. Clinical data of age, sex, site, initial symptoms, surgery extent, postoperative complications including recurrence, follow-up period, and current patient status were collected for analysis. Five-year cumulative survival rate was obtained using Kaplan-Meier method. At initial diagnosis, 22 patients were in the early stages (stage I: 15; stage II: 7) and 9 patients were in the advanced stages (stage III: 1; stage IV: 8). Lymph node metastasis was present in 5 patients and distant metastasis in 2. Of the 31 patients, 4 patients died (stage II: 1, stage IV: 3) during the follow-up period. Early-stage patients showed 100% 5-year estimated cumulative survival rate, whereas the advanced-stage patients showed 5-year estimated survival rate of 53.6% (p=.006). The overall survival rate of all enrolled patients was 90.3%. Although 5-year estimated disease-free survival rate of stage I was 100.0%, that for stage II was low at 30.0% because of considerable recurrences. The results of this multicenter study suggest that more aggressive treatment modality, including adjuvant therapy, is necessary for patients with CEAC with Pittsburgh stage II or more.

Reliability of Oronasal Fistula Classification.

Oronasal fistula is an important complication of cleft palate repair that is frequently used to evaluate surgical quality, yet reliability of fistula classification has never been examined. The objective of this study was to determine the reliability of oronasal fistula classification both within individual surgeons and between multiple surgeons. Using intraoral photographs of children with repaired cleft palate, surgeons rated the location of palatal fistulae using the Pittsburgh Fistula Classification System. Intrarater and interrater reliability scores were calculated for each region of the palate. Eight cleft surgeons rated photographs obtained from 29 children. Within individual surgeons reliability for each region of the Pittsburgh classification ranged from moderate to almost perfect (κ = .60-.96). By contrast, reliability between surgeons was lower, ranging from fair to substantial (κ = .23-.70). Between-surgeon reliability was lowest for the junction of the soft and hard palates (κ = .23). Within-surgeon and between-surgeon reliability were almost perfect for the more general classification of fistula in the secondary palate (κ = .95 and κ = .83, respectively). This is the first reliability study of fistula classification. We show that the Pittsburgh Fistula Classification System is reliable when used by an individual surgeon, but less reliable when used among multiple surgeons. Comparisons of fistula occurrence among surgeons may be subject to less bias if they use the more general classification of "presence or absence of fistula of the secondary palate" rather than the Pittsburgh Fistula Classification System.

Incidence of Palatal Fistula at Time of Secondary Alveolar Cleft Reconstruction

We aim to establish consistent time points for evaluating palatal fistula incidence to standardize reporting practices and clarify prospective literature. An institutional retrospective chart review was conducted on 76 patients with unilateral or bilateral complete cleft lip and palate who underwent secondary alveolar bone grafting between 2006 and 2015. Early fistula incidence rates were reported prior to maxillary expansion, and late fistula rates were reported at the time of alveolar bone grafting. Fistula recurrence rates after primary repair were also measured. We found an early fistula incidence rate of 20% (n = 15) and a late fistula rate of 55% (n = 42) at the time of secondary ABG. Fistulae recurred after initial repair in 43% of cases. Fistulae were classified using the Pittsburgh Classification System as type III (33%), type IV (60%), or type V (7%). The presence of a bilateral cleft ( P = 0.01) and history of early fistula repair ( P < 0.01) were associated with late fistula incidence in a univariate analysis. In a logistic regression model, only early fistula repair was associated with late fistula incidence (OR = 17.17) and overall likelihood of recurrence (OR = 70.89). Early fistulae should be reported prior to orthodontic expansion of the maxillary arch. Late fistulae should be reported at the time of ABG, following palatal expansion. Patients who develop an early fistula after palatoplasty are likely to experience recurrent fistula formation.

Oral Health-Related Quality of Life and Self-Rated Speech in Children With Existing Fistulas in Mid-Childhood and Adolescence.

To report the associations of oro-nasal fistulae on the patient-centered outcomes oral health-related quality of life and self-reported speech outcomes in school aged-children. Prospective, nonrandomized multicenter design. Six ACPA-accredited cleft centers. Patients with cleft palate at the age of mixed dentition. None. Prevalence of fistula and location of fistula (Pittsburgh Classification System). Patients were placed into one of three groups based on the following criteria: alveolar cleft present, no previous repair (Group 1); alveolar cleft present, previously repaired (Group 2); no congenital alveolar cleft (Group 3). Presence of fistula and subgroup classification were correlated to oral health-related quality of life (Child Oral Health Impact Profile [COHIP]) and perceived speech outcomes. The fistula rate was 5.52% (62 of 1198 patients). There was a significant difference in fistula rate between the three groups: Group 1 (11.15%), Group 2 (4.44%), Group 3 (1.90%). Patients with fistula had significantly lower COHIP scores (F1,1188 = 4.79; P = .03) and worse self-reported speech scores (F1,1197 = 4.27; P = .04). Group 1 patients with fistula had the lowest COHIP scores (F5,1188 = 4.78, P =.02) and the lowest speech scores (F5,1188 = 3.41, P = .003). Presence of palatal fistulas was associated with lower oral health-related quality of life and perceived speech among youth with cleft. The poorest outcomes were reported among those with the highest fistula rates, including an unrepaired alveolar cleft.

Tumor Stage-Related Role of Radiotherapy in Patients with an External Auditory Canal and Middle Ear Carcinoma.

PurposeThe purpose of this study was to evaluate the clinical outcomes of patients treated with radiotherapy (RT) for a carcinoma of the external auditory canal (EAC) and middle ear.Materials and MethodsThe records of 32 patients who received RT from 1990 to 2013 were reviewed retrospectively. The Pittsburgh classification was used to stage all the cancers (early stage, T1/T2 [n=12]; advanced stage, T3/T4 or N positive [n=20]). Twenty-one patients (65.6%) were treated with postoperative RT and 11 patients (34.4%) were treated with definitive RT. The median radiation doses for postoperative and definitive RT were 60 Gy and 64.8 Gy, respectively. Chemotherapy was administered to seven patients (21.9%).ResultsThe 5-year overall survival and disease-free survival rates for all patients were 57% and 52%, respectively. The disease control rates for the patients with early stage versus advanced stage carcinomawere 55.6% (5/9) and 50% (6/12) in the postoperative RT group and 66.7% (2/3) and 37.5% (3/8) in the definitive RT group, respectively. Overall, 15 cases (14 patients, 46.7%) experienced treatment failure; these failures were classified as local in four cases, regional in one case, and distant in 10 cases. The median follow-up period after RT was 51 months (range, 7 to 286 months).ConclusionPatients with early stage carcinoma achieved better outcomes when definitive RT was used. Advanced stage carcinoma patients experienced better outcomes with postoperative RT. The high rate of distant failure after RT, with or without surgery, reflected the lack of a consensus regarding the best therapeutic approach for treating carcinoma of the EAC and middle ear.

Cancer of the external auditory canal

Cancer of the external auditory canal is a rare tumour with an annual incidence of one per one million inhabitants. The objective of this study was to evaluate the 5-year overall survival and disease-free survival rates in a series of patients with carcinoma of the external auditory canal and to compare our results concerning the clinical presentation, management and survival with those of the literature. Ten patients were included in this retrospective, single-centre study over a 20-year period. Data concerning age, symptoms, imaging, TNM stage according to the Pittsburgh classification, histology, management, sequelae, recurrences and survival were recorded. The mean age of the patients of this series was 60.7 years. Seven patients had a squamous cell carcinoma. The other histological types were undifferentiated carcinoma, adenoid cystic carcinoma and neuroendocrine carcinoma. Staging was based on the Pittsburgh classification with one stage I, one stage III and eight stage IV tumours. Five-year overall survival rates were 100%, 50% and 0%, respectively. The mean 5-year overall survival rate was 35% and the mean 5-year disease-free survival rate was 24%. Carcinoma of the external auditory canal is a difficult diagnosis when the tumour does not present as a fungating mass protruding from the external auditory canal. The Pittsburgh classification was used for TNM staging of these tumours, allowing comparison of our results with those of the literature. The clinical findings and survival rates observed in this study are comparable to those reported in the literature. These tumours are associated with a poor prognosis on the basis of our results and published data.

Three-Layer Oronasal Fistula Repair With Sandwiched Mastoid Fascia Graft

Oronasal fistula can cause speech problems, hearing loss, velopharyngeal insufficiency, and social problems related with fetor oris and oronasal fluid leakage. The purpose of this study was to achieve 3-layer closure with autogenous mastoid fascia graft in a group of patients with recalcitrant oronasal fistulas.Sixteen patients, aged between 2 and 56 years (mean, 13.9 y), with recalcitrant palatal fistula were operated on and included into the study in a tertiary clinic. Nine patients had previous fistula repairs. The patients' mean follow-up period was 6.8 months.Fistula closure was obtained in 14 of 16 patients. All 2 failures had type IV + V fistulas according to Pittsburgh Classification. A 3-layer technique for the closure of fistulas with autogenous mastoid fascia graft allows three-dimensional repair of the defect without tension. Using mastoid fascia via postauricular sulcus incision is a good alternative regarding hiding incision scars and not requiring intraoperative repositioning.

Fermeture des fistules palatines séquellaires de fentes

Treatment of oronasal fistulae in cleft patients remains a surgical challenge because of its high failure rate. The authors report the results of an aggressive surgical technique using the total elevation of palatal mucoperiosteum, even for small fistulae. This approach was used on twelve consecutive patients, from five to 33 years of age, presenting with a Pittsburgh classification type IV palatal fistulae. The surgical procedure was total elevation of the hard palate mucoperiosteum starting from the dental sulcus combined with sealed double layer sutures. Clinical and photographical control was made at least 6 months after to detect a possible relapse. The success rate was 100%. No relapsing fistula was observed with follow-up ranging from 6 to 36 months. This technique allows wide exposure and safe closure of the nasal layer. It is simple and leaves no raw bone surface exposed and no additional scar. The authors think it can be used in all type IV fistulae less than 1cm wide. Several other surgical techniques have been described to close palatal fistulae: local turnover flaps, pedicled flaps from adjacent oral tissue, tongue flaps, tissue expansion, and even free flaps. Obturator prostheses have also been used. The technique we report, even if more aggressive, seems to be more reliable with fewer relapse and sequelae.

The problem of nodal disease in squamous cell carcinoma of the temporal bone

Conclusion: The prognosis of the clinically positive neck in temporal bone squamous cell carcinoma is bad and failures never occur in the neck but at the site of the primary. A clinically positive neck at presentation is a sign of aggressiveness of the primary and, in such cases, therapeutic neck dissection is to be performed. The role of elective neck dissection is partially supported by our results, but as prognosis worsens and survival drops when a positive node appears in the neck, elective dissection is still advocated. In our material, no recurrence was recorded in the neck, but it was the resection of the primary tumor that proved to be oncologically inadequate. This led to the conclusion that in cases of clinically positive necks a more extended and adequate approach to the primary is thus advocated. Objective: This work aimed to determine the prognostic role of positive neck nodes in squamous cell carcinoma of the temporal bone. Methods: We studied the homogeneous case material of 47 surgical cases of temporal bone squamous cell carcinoma, operated on between the years 1983 and 2004. All the cases were reviewed retrospectively, staged according to the Pittsburgh classification, and the follow-up was updated at June 2008 with MRI. The minimum follow-up was set at 3 years. Results: All the cases but two were treated with therapeutic or elective neck dissection. The neck was assessed both clinically and pathologically. The cases with clinically positive neck nodes at presentation had unfavorable prognosis. All the failures recurred at the site of the primary and never in the neck. The comparison of outcomes between the cases with clinically positive and negative necks showed a difference in survival. The rate of micrometastasis in the clinically negative neck was investigated as well. Our results indicate that though the necessity of elective neck dissection is still controversial, the bad prognostic value of the clinically positive neck is such to support the indication to treat the clinically negative neck.

Prognostic factors for squamous cell carcinoma of the temporal bone: extensive bone involvement or extensive soft tissue involvement?

Conclusion: Extensive bone erosion correlated with a worse prognosis of the squamous cell carcinoma (SCC) of the temporal bone but extensive soft tissue involvement did not correlate with prognosis in this study. Resectability of the tumor seems to be major prognostic factor of temporal bone SCC. Objective: Prognostic factors for SCC of the temporal bone were evaluated regarding initial clinical symptoms and radiographic imaging. Patients and methods: Clinical symptoms of the patients with primary SCC of the external auditory canal (EAC) or middle ear (ME) were reviewed based on medical records. Correlation of clinical symptoms and cancer severity staging using the modified Pittsburgh classification was analyzed, along with disease-specific survival (DSS). Results: Sixteen patients with primary SCC of the EAC (n=13) or ME (n=3) were included in the study population. DSS was not influenced by whether a hearing disturbance or otalgia was noted at the first medical examination. Extended bone involvement identified with imaging studies significantly correlated with worse prognosis (p<0.05). Prognoses of patients without extensive bone erosion were good, and extensive (≥0.5 cm) soft tissue involvement did not correlate with prognosis in this study. Prognoses of patients with insufficient bone resection or no surgery were significantly poor (p<0.01).

Squamous Cell Carcinoma of the External Auditory Canal

This study aims to analyze the treatments, prognostic variables, and outcomes of patients with squamous cell carcinomas (SCC) of the external auditory canal (EAC) and middle ear treated in our department over a 15-year period. A retrospective analysis of 19 patients treated in our department between 1990 and 2006. The patients were staged according to the Pittsburgh classification. Patients were treated with either a lateral (LTBR) or an extended temporal bone resection (total or subtotal). Parotidectomy was performed in patients with suspected clinical or radiological invasion and postoperative radiotherapy was the adjuvant treatment in most patients in advanced stages. The overall 5-year survival was 37%. There were no patients in stage I. The survival rates were 100%, 25%, and 16% for stages II, III, and IV, respectively. Facial nerve paralysis (p = 0.007) and lymph node involvement (p = 0.006) were associated with decreased survival rates. SCC of the temporal bone are rare but have a poor prognosis. Lymph node involvement and facial nerve palsy are associated with a poorer outcome. These tumors must initially be treated radically, for which an early diagnosis is important.

Primary Tumours of the External Auditory Canal. Our Experience in 34 Patients

Malignant neoplasms of the external auditory canal (EAC) and middle ear are rare but have a poor prognosis. The aim of this study is to identify the variables associated with worse prognosis.Thirty-four patients were treated in our department between 1990 and 2006 for EAC and middle ear tumours. The patients were staged according to the 1990 Pittsburgh classification. In most cases, surgery was followed by post-operative radiotherapy.The overall disease-free survival was 49 % after 5 years. It reached 87 % in stages I and II, whereas the survival for stages III and IV was 21 % (P=.001). Pre-operative facial nerve paralysis (P=.03), lymph node metastasis (P=.01) and dural extension (P=.02) were associated with decreased survival rates.In carcinomas of the EAC and middle ear, lymph node involvement, facial nerve palsy, and dural extension were associated with a poorer outcome. For tumours in advanced stages, new therapeutic protocols should be evaluated.

Immunodeficiency-related lymphoproliferative disorders: prospective data from the United Kingdom Children's Cancer Study Group Registry.

Clinical data and biological samples were prospectively collected in 42 children with lymphoproliferative disease (LPD) secondary to organ/bone marrow transplant-related immunosuppression (30: 11 liver, 10 heart/lung, 8 kidney and 1 bone marrow), other drug-induced immunosuppression (2), congenital immunodeficiency (8) or human immunodeficiency virus (HIV)-related immune dysfunction (2). Ages ranged from 10 months to 17 years and there were 15 girls. Pathology was centrally reviewed and showed polymorphic features in 5 cases, monomorphic in 23, mixed pattern in 5 patients and 9 other types. Using the Revised European-American Classification of Lymphoid Neoplasms, 5 were B lymphoblastoid, 24 were high-grade B and 14 were other subtypes. Using the Pittsburgh classification, 9 were lymphadenopathic, 10 were systemic, 25 were lymphomatous and, with the Murphy grouping for non-Hodgkin's lymphoma (NHL), 10 were localized and 32 non-localized. Twenty-four out of 38 evaluable cases were Epstein-Barr virus positive. Thirty-five patients were evaluable for clonality; 24 were monoclonal and 11 were polyclonal. Reduced immunosuppression in solid organ transplant patients resulted in resolution of disease in 14/24, which was sustained in 11. Nineteen patients received chemotherapy, 14/18 evaluable responded, which was sustained in 8 cases. Seven out of 29 solid organ transplant and 10/13 other immune-deficient patients died. In the largest group of patients, solid organ transplants, no significant clinical or biological characteristics that predicted outcome were identified. In the transplant group close monitoring of response during reduction in immunosuppression is essential and the early use of B NHL chemotherapy may be effective.