Can the Shriners Hospital Upper Extremity Evaluation (SHUEE) Detect Change in Dynamic Position and Spontaneous Function of the Upper Limb in People With Hemiplegic Cerebral Palsy?

BACKGROUND: The Shriners Hospital Upper Extremity Evaluation (SHUEE) is a video-based measure designed to assess upper extremity function in people with cerebral palsy (CP). The SHUEE completes both dynamic positional analysis (DPA; position during functional activities) and spontaneous functional analysis (spontaneous use of the involved limb). Although the SHUEE has been suggested as a measure for planning upper limb interventions and evaluating outcomes, limited evidence of its ability to detect change exists. Thus, this study aimed to describe responsiveness of the SHUEE to detect change after orthopaedic surgery. METHODS: In this Institutional Review Board-approved retrospective cohort study, we identified children with CP who were administered SHUEE on≥2 encounters. We formed pairs of initial and follow-up visits between temporally adjacent visits. Pairs were assigned to a surgery or non-surgery group based on intervening upper limb orthopaedic surgery. We compared differences in baseline SHUEE scores between groups and differences in temporally adjacent SHUEE scores within groups using Welch unequal variances t tests and paired t tests, respectively. RESULTS: Nineteen people (7 female) with hemiplegic CP had≥2 SHUEE assessments; Manual Ability Classification System levels I (3), II (8), III (7), IV (1); Gross Motor Function Classification System levels I (10), II (7), IV (2); mean age at baseline 11.9 (5.1 to 19.1) years; and follow-up at 13.4 (5.5 to 19.7) years. Six people had≥2 visits leading to 14 surgical pairs and 10 non-surgical pairs. At baseline, DPA of the wrist and forearm were significantly lower in the surgical group ( P <0.05). At follow-up, no significant difference between the groups existed in DPA measures ( P >0.05). After surgical intervention, there was a significant change in overall and wrist DPA ( P <0.05). CONCLUSIONS: The DPA measures demonstrated responsiveness to expected positional changes in the arm after orthopaedic surgery in people with CP. The SHUEE was useful in identifying abnormal segmental alignment pre-surgically and documenting changes in alignment postoperatively. As orthopaedic surgery does not address limb neglect or bimanual ability, spontaneous functional analysis scores were as expected-unchanged. LEVEL OF EVIDENCE: Level III, retrospective cohort study.

Walking activity after multilevel orthopedic surgery in children with cerebral palsy.

AIM: To determine how surgical burden and preoperative factors affect the recovery of walking activity after multilevel orthopedic surgery (MLS). METHOD: In this retrospective study, inclusion criteria were a diagnosis of cerebral palsy, MLS, and walking activity monitoring using a StepWatch device within 12 months pre-MLS and 24 months post-MLS. The outcome measure was total mean strides per day normalized to age and Gross Motor Function Classification System level. Pre- and postoperative walking activity were compared using unpaired t-tests; the effects of preoperative predictors and surgical burden on the recovery of walking activity were evaluated using regression analysis. RESULTS: Participants included 178 children (mean age 12 years 10 months [SD 8 years 7 months; range 4-20 years]; 91 males, 87 females). On average, children returned to baseline walking activity 3 months after low-burden surgery and 1 year 2 months after high-burden surgery. Postoperative walking activity was higher for children who had surgery at a younger age and those with a higher preoperative mobility function. INTERPRETATION: The burden of MLS was found to be inversely related to the time to recovery of postoperative walking activity. These findings provide evidence to help clinicians set expectations for return to function post-MLS. Further study is necessary to investigate the impact of postoperative factors on walking activity recovery. WHAT THIS PAPER ADDS: High-burden surgeries lead to longer recovery than low-burden surgeries. Younger children recover walking activity faster after multilevel orthopedic surgery. Children with high preoperative mobility function recover walking activity faster after surgery.

OBJETIVO: Determinar como a carga cirúrgica e os fatores pré-operatórios afetam a recuperação da atividade de caminhada após cirurgia ortopédica multinível (MLS). MÉTODOS: Neste estudo retrospectivo, os critérios de inclusão foram um diagnóstico de paralisia cerebral, MLS e monitoramento da atividade de caminhada usando um dispositivo Step Watch dentro de 12 meses pré-MLS e 24 meses pós-MLS. A medida de resultado foi o total de passos médios por dia normalizados para idade e nível do Sistema de Classificação da Função Motora Grossa. A atividade de caminhada pré e pós-operatória foi comparada usando testes t não pareados; os efeitos dos preditores pré-operatórios e da carga cirúrgica na recuperação da atividade de caminhada foram avaliados por meio de análise de regressão. RESULTADOS: Os participantes incluíram 178 crianças (idade média de 12 anos e 10 meses [DP 8 anos e 7 meses; intervalo de 4 a 20 anos]; 91 meninos, 87 meninas). Em média, as crianças retornaram à atividade de caminhada inicial 3 meses após a cirurgia de baixa carga e 1 ano e 2 meses após a cirurgia de alta carga. A atividade de caminhada pós-operatória foi maior para crianças que foram operadas em idade mais jovem e aquelas com maior função de mobilidade pré-operatória. INTERPRETAÇÃO: A carga de MLS foi inversamente relacionada ao tempo de recuperação da atividade de caminhada pós-operatória. Esses achados fornecem evidências objetivas para ajudar os médicos a definir as expectativas de retorno à função pós-MLS. Mais estudos são necessários para investigar o impacto dos fatores pós-operatórios na recuperação da atividade de caminhada.

Changes in foot posture evaluated with dynamic pedobarography over the course of childhood in ambulatory youth with cerebral palsy.

Purpose: Foot deformities are prevalent in children with cerebral palsy, but there is limited research on the progression of foot posture during growth. Our study aimed to evaluate the change in dynamic foot posture in children with cerebral palsy. Methods: Children with cerebral palsy, aged 17-40 months, were recruited to participate in this Institutional Review Board-approved prospective longitudinal study by having serial foot posture evaluations. The coronal plane index and foot segmental impulses were measured with dynamic pedobarography. Data were compared between children stratified by Gross Motor Function Classification System level and typically developing children using serial Welch's t-tests across time with Holm correction for multiple comparisons. Results: In total, 33 children (54 limbs) were included in the analysis (21 bilateral and 12 unilateral; Gross Motor Function Classification System: I-13, II-14, III-4, IV-2. Children completed 16.9 (± 4.4) evaluations (initial age 2.9 (± 0.7) and final age 18.6 (± 1.7) years)). Early valgus foot posture normalizes in children at Gross Motor Function Classification System levels I/II and persists in children at levels III/IV who do not have foot surgery. For most young children, foot posture development is variable. Conclusion: Foot posture in young children with cerebral palsy begins in valgus and tends to normalize in youth who walk without an assistive device. Conservative management of foot deformity is recommended in early childhood. Level of evidence: Level II, prognostic study.

Extrathoracic metastases of thymic origin: a review of 35 cases.

Thymic tumors are categorized as types A, AB, B1, B2, B3, and thymic carcinoma under the World Health Organization (WHO) classification. Thymomas are typically slow growing tumors that predominantly involve the surrounding structures through direct invasion, while thymic carcinomas tend to be more aggressive. A significant number of patients are asymptomatic and can present with metastases as the first presentation. The exact incidence of extrathoracic metastases from thymoma is not known. This study describes a series of 35 cases of histologically documented metastatic thymomas and thymic carcinomas at extrathoracic sites. These cases were classified according to the current World Health Organization (WHO) classification criteria, and we present their clinical data as well as discuss the differential diagnoses of these lesions. Our study shows that all types of thymic tumors, regardless of histologic type, can be associated with invasion and metastases to thoracic and extrathoracic sites.

The change in sagittal plane gait patterns from childhood to maturity in bilateral cerebral palsy.

BACKGROUND: The longitudinal stability of sagittal gait patterns in diplegic cerebral palsy (CP), stratified using the Rodda classification, is currently unknown. RESEARCH QUESTION: What is the trajectory of sagittal plane gait deformities as defined by the Rodda classification in a large cohort treated with orthopedic surgery guided by gait analysis? METHODS: A retrospective study utilized gait analysis to evaluate sagittal gait parameters before age 8 and after age 15 years. Individual limbs were categorized at each time point according to the Rodda classification based on mean sagittal plane knee and ankle angle during stance. Welch's t-tests compared gait variables from early childhood with maturity and examined changes associated with plantarflexor lengthening surgery. RESULTS: 100 youth with CP were evaluated twice: at a mean age of 5.49 ± 1.18 and 19.09 ± 4.32 years, respectively. Gross Motor Function Classification System distribution at maturity was I (10.5 %), II (55.2 %), III (28.6 %), and IV (5.7 %). At the initial visit, most limbs were in either true equinus (30 %) or jump-knee gait (26.5 %). At maturity, crouch gait (52.5 %) was the most common classification, of which 47.6 % were mild (1-3 standard deviations from age-matched norm; 21°-30°) and 52.4 % moderate or severe. For the entire cohort, at initial and final visits, respectively, mean knee flexion in stance was 26.8°±14.8° and 25.9°±11.4° (p = 0.320), ankle dorsiflexion in stance increased from -0.3°±11.5° to 9.0°±6.0° (p < 0.001), and passive knee flexion contracture was -2.3°±7.0° and -3.9°±8.0° (p = 0.043). In children who started in true equinus, apparent equinus, and crouch, there was no difference in stance phase knee flexion at maturity between those who underwent plantarflexor lengthenings versus those who did not (p > 0.18). SIGNIFICANCE: The trend in this cohort was toward crouch with increased stance phase ankle dorsiflexion from early childhood to maturity. Plantarflexor lengthenings were not a significant factor in the progression of stance phase knee flexion.

WHO types A and AB thymomas: not always benign.

The 2004 WHO classification of thymic tumors recognizes five major subtypes of thymomas and thymic carcinoma. Subtypes A and AB thymomas are purported to be benign neoplasms, although prior studies have suggested a potential for malignant behavior. The purpose of this study was to assess the clinical behavior of A and AB thymomas identified from a large institutional pathologic database. A retrospective slide review of 500 thymic epithelial tumors identified 71 (∼ 14%) cases of types A and AB thymomas. Clinical history and follow-up information were obtained through retrospective chart review. There were 38 and 33 cases of types A and AB thymomas, respectively. Complete follow-up data were available in 37 (52%) cases. Eighteen (49%) patients (type A, n=9 and type AB, n=9) had evidence of recurrent/metastatic disease at an average of 62 months (range from 6 to 244 months) after initial diagnosis. Survival curves for patients with types A and AB thymomas, with and without recurrences, show a statistically significant difference (P=0.001 and 0.005, respectively). Analysis of this large cohort confirms the potential for subtypes A and AB thymomas to show malignant behavior. Long-term clinical monitoring, therefore, appears to be justified in these cases. This study also shows the poor correlation between the WHO classification and tumor behavior.

Widespread cutaneous metastases from acinic cell carcinoma 20 years after primary presentation.

Acinic cell carcinoma is a rare, low-grade malignant salivary gland neoplasm. Approximately, 20% of patients experience local recurrence and 10% distant metastasis, often many years after initial presentation. We present a case of widespread cutaneous metastasis from acinic cell carcinoma arising in a 59-year-old woman with a history of acinic cell carcinoma of the parotid gland 20 years prior to her cutaneous disease. To our knowledge, is the first report of widespread cutaneous metastasis from acinic cell carcinoma.

Novel GALNT3 mutations causing hyperostosis-hyperphosphatemia syndrome result in low intact fibroblast growth factor 23 concentrations.

CONTEXT: Hyperostosis-hyperphosphatemia syndrome (HHS) is a rare metabolic disorder characterized by hyperphosphatemia and localized hyperostosis. HHS is caused by mutations in GALNT3, which encodes UDP-N-acetyl-alpha-D-galactosamine:polypeptide N- acetylgalactosaminyltransferase 3. Familial tumoral calcinosis (TC), characterized by ectopic calcifications and hyperphosphatemia, is caused by mutations in the GALNT3 or fibroblast growth factor 23 (FGF23) genes. OBJECTIVE: Our objective was to identify mutations in FGF23 or GALNT3 and determine serum FGF23 levels in an HHS patient. DESIGN: Mutation detection in FGF23 and GALNT3 was performed by DNA sequencing, and serum FGF23 concentrations were measured by ELISA. PATIENTS OR OTHER PARTICIPANTS: A 5-year-old French boy with HHS and his family members participated. RESULTS: The patient presented with painful cortical lesions in his leg. Radiographs of the affected bone showed diaphyseal hyperostosis. The lesional tissue comprised trabeculae of immature, woven bone surrounded by fibrous tissue. Biochemistry revealed elevated phosphate, tubular maximum rate for phosphate reabsorption per deciliter of glomerular filtrate, and 1,25-dihydroxyvitamin D levels. The patient was a compound heterozygote for two novel GALNT3 mutations. His parents and brother were heterozygous for one of the mutations and had no biochemical abnormalities. Intact FGF23 level in the patient was low normal, whereas C-terminal FGF23 was elevated, a pattern similar to TC. CONCLUSION: The presence of GALNT3 mutations and elevated C-terminal, but low intact serum FGF23, levels in HHS resemble those seen in TC, suggesting that HHS and TC are different manifestations of the same disorder. The absence of biochemical abnormalities in the heterozygous individuals suggests that one normal allele is sufficient for secretion of intact FGF23.

Diagnostic utility of thymic epithelial markers CD205 (DEC205) and Foxn1 in thymic epithelial neoplasms.

Foxn1 and CD205 (DEC205) are novel thymic epithelial markers that are important for thymic organogenesis and the positive selection process for thymocytes, respectively. These markers were immunohistochemically applied to a total of 77 cases of thymic epithelial neoplasms comprised of 58 cases of thymomas, 17 cases of thymic carcinomas, and 2 cases of thymic neuroendocrine carcinomas. Foxn1 was diffusely expressed in nuclear staining in all cases of type B thymoma and all but 1 case of type A thymoma, whereas the expression was generally focal in thymic carcinoma (76%). The expression was identified in all cases of mixed AB thymoma, with the expression in type A component being more variable than the one in type B component. CD205 cytoplasmic expression in the form of coarse granular staining with membranous accentuation was strong and diffuse in all cases of type B thymoma (100%), and a majority of type A thymoma (89%), and focal with variable intensity in thymic carcinoma (59%). Mixed AB thymoma demonstrated diffuse expression in type B component (100%), and variable expression in type A component (94%). Neither Foxn1 nor CD205 was expressed in 2 cases of thymic neuroendocrine carcinoma. Foxn1 was focally expressed in 13% of cutaneous squamous cell carcinoma and completely negative in cutaneous basal cell carcinoma, whereas it was completely negative in squamous cell carcinoma from head and neck, esophagus and uterine cervix, and normal tissue and malignant neoplasms from all other organs other than thymus. CD205 was expressed in 4% of nonsmall cell carcinomas of lung, 27% of squamous cell carcinoma of head and neck, and 10% of squamous cell carcinoma of esophagus, but the staining pattern was different from that of thymic epithelial neoplasm and was characterized by rather homogeneous and amorphous quality without granularity or membranous reaction. CD205 was expressed in myeloid dendritic cells of various organs and tissues as well. Foxn1 is a sensitive and specific marker for thymoma and thymic carcinoma, and it appears to be superior to CD5 and CD117 for the diagnosis of thymic carcinoma. CD205 is a sensitive and specific marker for thymoma but its sensitivity to thymic carcinoma is lower than CD5 and CD117.

Malignant Sertoli cell tumors of the testis: a study of 13 examples of a neoplasm frequently misinterpreted as seminoma.

The distinction of Sertoli cell tumors from seminoma is critical to ensure proper treatment. Although usually straightforward, we highlight herein 13 malignant Sertoli cell tumors of the testis with light microscopic features that mimicked seminoma. All of the cases were received in consultation, 10 with submitting diagnoses of seminoma, usually of classic type, but three cases of spermatocytic type. Patients ranged from 15 to 80 years of age (median 37 years); all presented with testicular masses. The tumors were typically firm, white to yellow-tan, and often had foci of hemorrhage. The dominant microscopic pattern was nested or sheet-like, with some tumors having secondary patterns of trabeculae-solid tubules, hollow tubules, and pseudofollicles. Tumor cells were polygonal with conspicuous clear cytoplasm in 12 cases; the cytoplasm was focally eosinophilic in 10 cases, but this was never conspicuous. Nine tumors had cytoplasmic vacuoles, and three of four that were investigated stained for intracytoplasmic glycogen. Nuclei were small (5) to medium-sized (8), round-to-oval (13), and vesicular with irregular contours (11). Nucleoli were present in 11 tumors (six small; five large). Stromal fibrosis (12) and lymphoid infiltrates (10) were conspicuous, and tumor necrosis (11) and vascular invasion (8) also were seen. Mitotic figures ranged from <1 to 21/10 high power fields (HPF) (median 1/10 HPF). Staining for inhibin-alpha, epithelial membrane antigen, and cytokeratin (AE1/AE3) was positive in four of four, six of six, and three of six cases, respectively; placental alkaline phosphatase was negative in all five tumors investigated. The nested growth pattern, prominence of clear cells, lymphoid infiltrate, inconspicuous tubular differentiation, cytoplasmic glycogen, and prominent nucleoli caused these tumors to be mistaken for seminomas. The smaller, less pleomorphic nuclei of Sertoli cell tumors, their lower mitotic rate, and the absence of intratubular germ cell neoplasia are helpful differential features. Immunohistochemistry is a useful adjunct in confirming the diagnosis of Sertoli cell tumor, but only if the overlapping features are appreciated by conventional microscopy and the diagnosis of Sertoli cell tumor included in the differential.

Seminomas with exclusive intertubular growth: a report of 12 clinically and grossly inconspicuous tumors.

Intertubular growth in seminoma is characterized by seminoma cells, either singly or in small clusters, between preserved seminiferous tubules. It is a common, although focal, pattern in many seminomas where it is admixed with the usual sheet-like and nested arrangements and does not pose any diagnostic problems in such cases. We describe, in contrast, the clinicopathologic features of 12 cases with exclusively intertubular growth and which were typically diagnostically problematic. The 12 patients lacked overt clinical signs of a primary testicular mass. Three presented with infertility, 2 with cryptorchidism, 2 with metastases, 1 with pain and testicular atrophy, and the presentation was unknown in 4. On gross examination, no mass was apparent in 9 cases with available data, but ill-defined firm areas or foci of whitish-brown discoloration were occasionally noted. Microscopically, the process was characterized by individual, dispersed tumor cells or small clusters of cells growing between the seminiferous tubules. The tumor cells were often obscured by a lymphocytic infiltrate or, less commonly, nodules of hyperplastic Leydig cells. Common associated findings were tubular atrophy with sclerosis and thickening of tubular basement membranes and intratubular germ cell neoplasia, unclassified type. Immunostains against placental-like alkaline phosphatase and c-KIT (CD117) highlighted the seminoma cells in all cases examined. In pure form, intertubular seminoma is both clinically and pathologically inconspicuous and may be misdiagnosed as atrophy, scar, or orchitis.

Cystic trophoblastic tumor: a nonaggressive lesion in postchemotherapy resections of patients with testicular germ cell tumors.

Cystic trophoblastic tumor (CTT) is an uncommon lesion that is usually seen after chemotherapy in patients with testicular germ cell tumors. Its clinical significance has not been well studied. We identified 17 patients with CTT in retroperitoneal lymph node dissections (RPLNDs) after cisplatin-based chemotherapy for testicular germ cell tumors. None had other forms of persistent germ cell tumor except for teratoma, and no patient received additional chemotherapy after RPLND. At the time of RPLND, 7 patients were known to have had normal serum levels of beta-subunit of human chorionic gonadotropin (beta-hCG), whereas 5 had relatively mild elevations (1.6-165 mIU/mL, median, 8.0 mIU/mL). The CTTs consisted of circumscribed, small cysts, usually multifocal, lined by mostly mononucleated trophoblast cells with abundant eosinophilic cytoplasm, often with smudged nuclei and showing only infrequent mitotic figures. Although the epithelial lining was often stratified to several layers in thickness or formed intracystic papillary tufts, solid proliferations of trophoblast cells within the stroma were absent, as were clearly biphasic admixtures of mononucleated and multinucleated trophoblast cells. The cysts were either empty or contained fibrinoid material and were set in a hypocellular, fibrous stroma with adjacent teratoma. Stains for hCG highlighted rare cells. On follow-up of 15 patients, 11 were disease free (mean, 80 months). Three recurred with serum alpha-fetoprotein elevations at 25, 31, and 107 months, respectively, and one with beta-hCG elevation at 2 months. The latter patient, however, also had unresected mediastinal tumor postchemotherapy. We conclude that the finding of CTT in postchemotherapy resections does not warrant additional chemotherapy. Its clinical significance appears similar to that of residual teratoma.

Tyrosine kinase receptor expression in thymomas.

PURPOSE: Promising new therapies for neoplasia include tyrosine kinase receptor antagonists. Tyrosine kinase oncogenes present an appealing anti-tumor drug target since they play an integral role in a variety of cellular responses including cell proliferation and differentiation. We previously demonstrated a high rate of epidermal growth factor receptor (EGFR) expression in advanced-stage thymic epithelial tumors. More recently, we have examined c-KIT (CD117) expression in a similar series of tumors. METHODS: Tumor from 35 patients seen at our institution for treatment of advanced-stage thymoma was available. Twenty thymomas and 15 thymic carcinomas were assessed for c-KIT expression. Tissue sections of tumor were stained immunohistochemically with anti-c-KIT (Oncogene). Either cytoplasmic or membrane staining was considered positive. Appropriate controls were performed. Positive staining for c-KIT was present in 12 tumors (11 thymic carcinomas and 1 thymoma). RESULTS: In distinction to EGFR, c-KIT is expressed more commonly in thymic carcinomas (73% of carcinomas) than in thymoma (5% of thymomas). CONCLUSIONS: An EGFR negative/c-KIT positive staining pattern is typical of thymic carcinoma, whereas thymomas are generally EGFR positive/c-KIT negative. Possible therapeutic implications of these observations remain to be determined.

Prognostic indicators after surgery for thymoma.

BACKGROUND: We undertook a 20-year retrospective institutional study to investigate prognostic indicators after surgery for thymoma. METHODS: From 1989 to 2009, 83 patients underwent surgical resection of thymoma or thymic carcinoma at our institution. Twelve of these patients were determined to have either World Health Organization type C disease or Masaoka stage IV-B disease and were excluded from analysis. The remaining 71 patients were reviewed. RESULTS: The majority of patients in this series were female 64.7% (n=46) with an overall average age of 51.0 years. The distribution of Masaoka stages I, II, III, and IV-A was 40.8% (n=29), 19.7% (n=14), 18.3% (n=13), and 21.1% (n=15), respectively. Thirteen of the 28 (46.2%) patients who presented with stage III or IV-A disease received preoperative chemotherapy. After a mean follow-up of 66 months (range, 6 to 241 months), 54 (75.3%) patients are alive and well while six are alive with disease. Eleven (16.0%) patients have died, but only 3 (4.3%) of these patients died of thymoma. The overall disease-specific survival was 97% and 89% at 5 and 10 years. Of the variables analyzed, only age was predictive of overall survival (p=0.03). Masaoka stages I to III as compared with stage IV-A was significantly predictive of disease-free survival (p<0.01). CONCLUSIONS: Long-term disease-specific survival can be expected not only after surgery for early stage thymoma but also after surgery for advanced disease, including patients with pleural metastases. However, patients who undergo surgery for stage IV-A disease have reduced disease-free survival. Late mortality due to secondary cancers and associated immunologic disorders was more frequent than mortality from thymoma in this series.

Spindle cell lipoma of the oral cavity.

Spindle cell lipoma is typically seen in the neck/trunk region of middle-aged and older men. Rare cases of oral spindle cell lipoma have been reported. An entity described as myxoid lipoma of the oral cavity has rarely been reported but appears to be more properly classified as spindle cell lipoma. We describe the largest series yet of oral spindle cell lipoma involving the tongue (4), buccal mucosa (1), floor of mouth (1), and lip (1). The patients (3M; 4F) ranged from 31 to 88 years old. All presented with mass lesions. All were circumscribed and composed of mature adipocytes admixed with bland spindled cells. In two cases the adipocytes appeared atrophic, imparting a pseudo-lipoblastic appearance. No true lipoblasts were seen and none had the characteristic vasculature of a myxoid liposarcoma or the characteristic hyperchromatic cells of well-differentiated liposarcoma. The stromal background of all cases contained characteristic wiry collagen and myxoid ground substance. The myxoid ground substance was prominent in four cases. Immunohistochemical stains for CD34 highlighted the bland spindle cells in all cases. The combination of the histologic features and the immunoreactivity for CD34 confirmed the diagnosis. Spindle cell lipoma should be considered in the differential diagnosis of oral cavity mesenchymal tumors.

Gastrointestinal stromal tumors: CT and MRI findings.

The objective of this study was to report the CT and MRI appearances of primary and metastatic gastrointestinal stromal tumor (GIST). The clinical and imaging findings of 31 patients with histological and immunohistochemical diagnosis of GIST were reviewed. The CT and MRI findings were assessed independently for size, location, enhancement characteristics, and pattern of metastatic disease. The tumors were of enteric (n=13), gastric (n=12), duodenal (n=2), and rectal (n=3) origin. In one case the primary site was the mesentery, without involvement of bowel. Primary tumors were typically exophytic (79%), larger than 5 cm (84%), and inhomogeneously enhancing (84%). Central necrosis of all tumors (37%) and aneurysmal dilation of enteric tumors (33%) were less common. Metastases were most commonly to mesentery (26%) or liver (32%). Less common findings were ascites (7%) and omental caking (3%). Liver metastases were hypervascular in 92% of patients and rapidly became cystic following therapy with imatinib mesylate (Gleevec; Novartis, East Hanover, NJ, USA). Lung metastases, bowel obstruction, vascular invasion, and significant lymphadenopathy were not seen in any patient. GISTs have some specific CT findings which could help differentiate them from other gastrointestinal tumors. Liver metastases became cystic following therapy, mimicking simple cysts. MRI was better than single-phase CT for assessing liver metastases, while CT was more sensitive for mesenteric metastases.

Peculiar cytoplasmic inclusions in oncocytic adrenal cortical tumors: an electron microscopic observation.

Two cases of an oncocytic adrenal cortical tumor that contained peculiar cytoplasmic crystalline inclusions in the tumor cells are presented. The patients were 49- and 72-year-old females without clinical and biochemical evidence of adrenal cortical or medullary dysfunction. The adrenal tumors weighed 80 and 200 g each. These crystalline inclusions were present in groups of longitudinal profiles or clusters of crossly cut aggregates. They appeared in clusters of membrane-bound columns. On longitudinal sections, they appeared as rigid rods of homogenous density measuring 36 nm in width, but when they were cut transversely their paracrystalline nature became apparent. They were composed of closely packed microtubules in rectangular blocks. The microtubules measured 12.5 nm with a hollow center measuring 4.2 nm. The inclusions were within the membrane-bound cisterna of rough-surfaced endoplasmic reticulum. The significance of these inclusions is not clearly understood; however, they have been seen only in adrenal cortical tumors and their presence may be helpful in the differential diagnosis of adrenal oncocytic tumors. One patient presented with a tumor in which gross and microscopic appearance was compatible with a pheochromocytoma. This case exhibited an oncocytic appearance and pronounced cellular pleomorphism. Ultrastructural studies were necessary to recognize the tumor cells as cortical cells. The tumor cells contained abundant mitochondria with tubular cristae, paranuclear parallel stacks of granular endoplasmic reticulum, and relatively prominent smooth endoplasmic reticulum. These features are typical of adrenocortical cells. In addition, frequent tumor cells contained the peculiar cytoplasmic inclusions herein described.

Prostate carcinoma presenting as multiple pulmonary nodules in an asymptomatic patient with a history of testicular nonseminomatous germ cell tumor.

Prostate carcinoma presenting initially as multiple pulmonary nodules in an asymptomatic patient without previous prostate carcinoma is unusual. Whether the incidence of prostate carcinoma is significantly increased in patients treated previously for germ cell tumors is unclear. We report such a patient, who responded to combination androgen blockade therapy.

Detection of pyloric metaplasia may improve the biopsy diagnosis of Crohn's ileitis.

BACKGROUND: Pyloric metaplasia (PYME) is seen frequently in ileal resections from patients with Crohn's disease (CD). It represents a nonspecific reparative reaction in intestinal ulcers. The presence of PYME in terminal ileum biopsies (TIbxs) of CD has not received much attention and has been considered a rare finding. STUDY: We reviewed 45 TIbxs from 45 adult patients with indisputable clinicopathologic diagnosis of CD. Endoscopic biopsies were obtained with an Olympus CIF 140 videoscope. The samples were fixed in 10% buffered formalin and were processed routinely. RESULTS: Pyloric metaplasia was identified in 10 TIbxs from 10 patients. Most of the metaplastic glands formed small aggregates in the mucosal base, and they were frequently noted below regenerating crypts and in the vicinity of granulation tissue and ulceration. CONCLUSIONS: In our material, 22.2% of the TIbxs revealed PYME. The single previous study with similar data showed an incidence of 2.27%. The difference could reflect the stage of the disease or the thoroughness of the endoscopic and pathologic examinations. Pyloric metaplasia, despite its lack of specificity, remains a sensitive indicator of persistent ulceration with inflammation. Because PYME is not as rare as previously thought, it should be carefully looked for in TIbxs to support the diagnosis of CD in the appropriate clinical setting.

Identical allelic losses in mature teratoma and other histologic components of malignant mixed germ cell tumors of the testis.

Teratomas of the testis in post-pubertal patients are histologically diverse tumors that often coexist with other types of germ cell tumors. Using laser capture microdissection and loss of heterozygosity analysis, we investigated the clonality of mature teratoma and its relationship to other components of malignant mixed germ cell tumors to gain potential insight into the histogenetic relationship of teratoma with other germ cell tumor components. All 16 patients had mature teratoma as one component of their mixed germ cell tumors. The other histological subtypes included immature teratoma, seminoma, embryonal carcinoma, yolk sac tumor, and choriocarcinoma. Laser-assisted microdissection was performed on the formalin-fixed, paraffin-embedded tissue. Polymerase chain reaction was used to amplify genomic DNA at specific loci on chromosome 1p36.2 (D1S508), 2q22-32 (D2S156), 9p21-22 (D9S162), 11p13 (D11S903), 12q22-23 (D12S1051), and 18q21 (D18S46). Fourteen of 16 (88%) cases showed allelic loss in one or more components of the mixed germ cell tumors. Fourteen of 16 mature teratomas showed allelic loss in at least one of six microsatellite polymorphic markers analyzed. The frequency of allelic loss in mature teratoma was 50% (7 of 14) with D1S508, 33% (5 of 15) with D2S156, 58% (7 of 12) with D9S162, 43% (6 of 14) with D11S903, 20% (3 of 15) with D12S1051, and 33% (5 of 15) with D18S46. Completely concordant allelic loss patterns between mature teratoma and all of the other germ cell tumor components were seen in 10 of 14 tumors in which mature teratoma showed loss of heterozygosity. Our data support the common clonal origin of mature teratoma with other components of malignant mixed germ cell tumors of the testis.