Glycogenic Hepatopathy: A Rare Hepatic Complication of Poorly Controlled Type 1 DM.

Glycogen hepatopathy (GH) is a rare complication of type 1 diabetes mellitus that leads to an abnormal accumulation of glycogen in the hepatocytes. The exact mechanism of GH remains unknown, but fluctuations in blood glucose and insulin levels play important roles in promoting glycogen accumulation. We report a case of a 16-year-old female diagnosed with poorly controlled type 1 diabetes mellitus with hepatomegaly and elevated liver enzymes. The patient experienced multiple admissions for diabetic ketoacidosis, and she also had celiac disease diagnosed 2 years previously based on serology and a duodenal biopsy. The laboratory analyses results were compatible with acute hepatitis, and the celiac serology was positive. Other investigations ruled out viral hepatitis and autoimmune and metabolic liver diseases. Ultrasound and computerized tomography (CT) scans of the abdomen revealed liver enlargement with diffuse fatty infiltration. A liver biopsy revealed the presence of abundant glycogen in the cytoplasm of the hepatocytes. PAS staining was strongly positive, which confirmed the diagnosis of GH. There were no features of autoimmune hepatitis or significant fibrosis. Duodenal biopsy results were consistent with celiac disease. Despite our efforts, which are supported by a multidisciplinary team approach that included a hepatologist, a diabetic educator, a dietitian, and an endocrinologist, we have encountered difficulties in controlling the patient's diabetes, and she persistently maintains symptomatic hepatomegaly and abnormal liver biochemistry. Given the patient's age, we assumed that these abnormalities were related to patient noncompliance. In conclusion, GH remains an under-recognized complication of type 1 DM that is potentially reversible with adequate glycemic control. The awareness of GH should prevent diagnostic delay and its implications for management and the outcome.

Immunohistochemistry of mammary Paget's disease. Cytokeratin 7, GATA3, and HER2 are sensitive markers.

OBJECTIVES: To investigate the expression of various immunohistochemical markers in Mammary Paget's disease (MPD) and MPD-associated breast carcinoma and to evaluate their value in establishing the diagnosis. METHODS: This retrospective descriptive study was carried out at King Faisal Specialist Hospital and Research Center and King Khalid University Hospital, Riyadh, Saudi Arabia. All MPD cases reported between January 2010 and June 2016 were selected from the surgical pathology records.  Immunohistochemical staining was carried out for cytokeratin 7 (CK7), GATA-binding protein 3 (GATA3), human epidermal growth factor receptor 2 (HER2), and estrogen, and progesterone receptors. Results: Twenty-two cases of MPD and 20 cases of MPD-associated breast carcinoma were included. CK7 was positive in 95% (21/22) cases of MPD and in all (20/20) cases of associated breast carcinoma. Similarly, GATA3 was expressed in 95% cases of both MPD (21/22) and associated breast carcinoma (19/20). Human epidermal growth factor receptor 2 was also overexpressed in 90% cases of MPD (20/22) and associated breast carcinoma (18/20). Estrogen stained positive in 27% cases of MPD (6/22) and 30% of cases of associated breast carcinoma (6/20). Progesterone receptors was not positive in any case of MPD; however, it was seen positive in 25% cases of MPD-associated breast carcinoma (5/20). CONCLUSIONS: CK7, GATA3, and HER2 are widely expressed in MPD and MPD-associated breast carcinoma. These markers can be used for the immunohistochemical confirmation of MPD including CK7-negative cases.

Molecular subtyping of mammary Paget's disease using immunohistochemistry.

OBJECTIVE: To evaluate the molecular subtypes of Mammary Paget's disease (MPD) and the associated breast carcinomas. METHODS: This retrospective study was carried out at King Khalid University Hospital and King Faisal Specialist Hospital, Riyadh, Saudi Arabia. Data from MPD patient cases from January 2010 to June 2016 were reviewed. The molecular subtypes were determined based on estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 (HER2) expression with immunohistochemical staining. The relative frequencies of the luminal A and B, HER2-enriched and basal-like molecular subtypes were calculated and compared for MPD and the associated breast carcinomas. Results: Among 22 patients with MPD, HER2-enriched was the most frequently occurring molecular subtype and was observed in 11 (50%) patients. Mammary Paget's disease was classified as basal-like in 5 (22.7%) patients, and luminal A and B were each detected in 3 (13.6%) patients. The molecular subtype of MPD corresponded with the subtype of the associated breast carcinoma in 18 out of 20 patients (90%). CONCLUSIONS: The HER2-enriched subtype is the most frequently occurring molecular subtype in MPD. The molecular subtype of the associated breast carcinoma is usually similar to that of MPD. The molecular subtypes vary between MPD associated breast carcinoma and overall breast carcinoma. The HER2-enriched subtype is the most frequently occurring subtype of MPD associated breast carcinoma, while luminal subtypes are more common in overall breast carcinoma.

Squamous Cell Carcinoma Arising in Zenker's Diverticulum: A Case Report and Review of the Literature.

The occurrence of squamous cell carcinoma (SCC) arising in a Zenker's diverticulum is a very rare incident. Complete excision of the diverticulum is considered as the procedure of choice for SCC in the pharyngeal pouch. Histopathological assessment of the pouch is the only modality to rule out SCC. Here, we report a case of a 71-year-old male with 20 years of history of Zenker's diverticulum, who recently presented with a history of weight and appetite loss. A barium swallow confirmed Zenker's pouch, the patient underwent diver-ticulectomy and cricopharyngeal myotomy; a histopathological examination of the specimen revealed a fungating mass of SCC within the pouch. This report highlights the suggestion of considering SCC not only in patients with a long history of Zenker's diverticulum but also when there is a clinical suspicion with new symptoms for a more aggressive management for diagnosis and complete excision of the pouch.

Endobronchial biopsy in the final diagnosis of chronic obstructive pulmonary disease and asthma: a clinicopathological study.

BACKGROUND: Asthma and chronic obstructive pulmonary disease (COPD) are chronic conditions with an increasing prevalence in developing countries. The evaluation of endobronchial biopsies has emerged as a tool to differentiate between both conditions via the measurement of the reticular basement membrane (RBM) thickness with various conclusions drawn from different studies. OBJECTIVES: Compare the thickness of the RBM between asthma and COPD and evaluate other histomorphological features in both groups. DESIGN: Prospective, descriptive and analytical. SETTING: University teaching hospital. PATIENTS AND METHODS: The study included patients with COPD and irreversible and reversible asthma with diagnosis based on clinical assessment, pulmonary function tests and high-resolution computed tomography scans. Endobronchial biopsies were obtained from all patients and, using a light microscope and a computerized image analyzer, the thickness of the reticular basement membrane was calculated in all patients. We also made a qualitative assessment of other histo-morphological features. MAIN OUTCOME MEASURES: Mean RBM thickness. SAMPLE SIZE: Thirty male patients. RESULTS: The mean RBM thickness in asthmatic patients was 8.9 (2.4) micro m. The mean RBM thickness in COPD patients was 5.3 (1.1) micro m. However, there was no thickening of the RBM in patients with reversible asthma. The RBM was significantly thicker in patients with irreversible asthma than in patients with COPD or reversible asthma. There were no significant differences in epithelial desquamation or metaplasia, mucosal or submucosal inflammation, the presence of eosinophils, submucosal glandular hyperplasia or submucosal smooth muscle hyperplasia between groups. CONCLUSIONS: The thickness of the RBM is the only reproducible histopathological feature to differentiate COPD from irreversible asthma. LIMITATIONS: The study included a limited number of patients. A qualitative approach was used to compare epithelial cell injury, inflammation, submucosal glandular and muscular hyperplasia. CONFLICT OF INTEREST: None.

Can computed tomography and carbon monoxide transfer coefficient diagnose an asthma-like phenotype in COPD?

BACKGROUND AND OBJECTIVE: Post-mortem and computed tomography (CT) studies indicated that emphysema is a feature of COPD even in the 'blue bloater/chronic bronchitis' type. We aim to test the hypothesis that the non-emphysematous patients are distinct from the main body of COPD and are more akin to asthmatic patients. METHODS: We studied 54 patients with COPD. Emphysema was measured by Goddard's visual scoring of CT scan and the carbon monoxide transfer coefficient (KCO). Bronchial biopsy was offered for thickness of basement membrane (BM) (≥7 µm) as a marker of remodelling in irreversible asthma. Spirometry was repeated after therapy with Budesonide/Formoterol for 1 year. RESULTS: The non-emphysematous phenotype were 24 of 54 patients (44%) by CT scan and 23 of 54 patients (43%) by KCO, showing agreement in 53 out of 54 patients. The non-emphysematous patients were younger, had higher forced expiratory volume in 1 s (FEV1 ) (median 61% vs 49.7%), greater prevalence of hypertrophy of nasal turbinates and higher serum IgE. The emphysematous phenotype had lower BMI and greater dyspnoea score. The BM was thickened in 11 of 14 and 0 of 10 patients in the non-emphysematous and emphysematous groups, respectively. Three patients without emphysema and a normal BM normalized their FEV1 upon receiving inhaled corticosteroid (ICS)/long-acting ß2 agonist (LABA). All the non-emphysematous improved their FEV1 after ICS/LABA (median = 215 mL). The median decline in the emphysematous was -65 mL. CONCLUSION: The non-emphysematous phenotype of COPD displays important features of asthma: clinical picture, histology and response to ICS. CT and KCO can predict spirometric response to ICS/LABA.

Differential diagnosis between pancreatic neuroendocrine and solid pseudopapillary neoplasms on endoscopic ultrasound-guided fine-needle aspiration. An immunohistochemical study.

OBJECTIVES: To evaluate the role of applying a limited panel of immunohistochemical stains on the cellblock preparation from samples obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) in the aim of differentiating solid pseudopapillary neoplasms (SPNs) from neuroendocrine tumors (NETs).  METHODS: We retrospectively retrieved all the EUS-FNAs of the pancreas that have a diagnosis of NET or SPN that were performed at 2 tertiary care hospitals in Riyadh, Kingdom of Saudi Arabia from May 2004 to December 2014. Diff-Quik, Papanicolaou, and Immunohistochemistry stains on cellblock preparations were performed.   RESULTS: Twenty cases were available (16 pancreatic neuroendocrine tumors (pNETs) and 4 SPNs). The pNETs were immunoreactive for synaptophysin, chromogranin A and CD56 while E-cadherin was diffusely to focally cytoplasmic positive. ß-catenin was negative or showed focal cytoplasmic immunoreactivity. In comparison, SPNs were positive for vimentin, CD10, CD-56, focally positive for progesterone receptors and synaptophysin, and revealed nuclear immunostaining for ß-catenin. They were negative for chromogranin A and E-cadherin.  CONCLUSION: Based on EUS-FNA samples, nuclear immunoreactivity for ß-catenin with loss of membranous immunostaining for E-Cadherin can potentially facilitate differentiating SPNs from pNETs.

Cytologic diagnosis of gastric submucosal lesions by endoscopic ultrasound-guided fine-needle aspiration: A single center experience in Saudi Arabia.

BACKGROUND AND AIMS: Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) sampling has become standard practice for the diagnosis of submucosal gastrointestinal (GI) lesions. The aim of this study was to determine the utility of EUS-guided FNA cytology in the diagnosis of deeply seated gastric mass lesions. MATERIALS AND METHODS: Thirteen patients with deeply seated gastric mass lesions were diagnosed by EUS-FNA. Adequate cytology material was present in all cases. Cell blocks were available in 10 cases. Surgical resections were performed in 8 cases. Immunohistochemical (IHC) studies were done on cell blocks in 9 cases and on 6 resected specimens. Seven cases has proved to be GI stromal tumors (GIST), in four of them, cell blocks were available, and resection for GIST was performed in 5 cases. IHC stains that were performed in cytology, as well as resection specimens, revealed similar results in each patient. CONCLUSION: EUS-FNA cytology, when combined with a histologic assessment of cell blocks provides accurate and efficient tissue diagnosis of a wide variety of deeply seated gastric mass lesions.

Utility of endobronchial ultrasound-guided-fine-needle aspiration and additional value of cell block in the diagnosis of mediastinal granulomatous lymphadenopathy.

BACKGROUND: Endobronchial ultrasound-guided transbronchial fine-needle aspiration is a minimally invasive technique for diagnosis of mediastinal lesions. Although most studies have reported the utility of EBUS-FNA in malignancy, its use has been extended to the benign conditions as well. OBJECTIVE: To evaluate the diagnostic yield and cytologic accuracy of endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-FNA) in cases of clinically and radiologically suspected granulomatous diseases. PATIENTS AND METHOD: From May 2010 to April 2015, 43 of 115 patients who underwent EBUS-FNA at one center for radiologically and clinically suspicious granulomatous lesions, and with no definite histological diagnosis, were included in this retrospective study. RESULTS: When the histological diagnosis was taken as the gold standard, the sensitivity of EBUS-FNA was 85% and specificity was 100% with the positive predictive value of 100. The combined diagnostic sensitivity of EBUS-FNA and transbronchial lung biopsy was 100%. In 4 cases, cell block provided an exclusive morphological diagnosis of sarcoidosis which was noncontributory by EBUS-FNA. CONCLUSION: Our study supports the use of EBUS-FNA, by virtue of being a safe, minimally invasive, and an outpatient procedure, in the diagnosis of granulomatous mediastinal lymphadenopathy, thereby obviating more invasive testing in a significant number of patients. Also, cell block provides additional data in the diagnosis in these benign mediastinal diseases.

Cytology findings in pancreatic heterotopia, a potential pitfall for malignancy: a case report and literature review.

Pancreatic heterotopia is a rare congenital disorder occurring at a variety of sites in the gastrointestinal tract. It is rarely symptomatic. Despite advances in diagnostic techniques, it still remains a challenge to the clinician to differentiate it from a neoplasm. Cytologic characteristics of pancreatic heterotopia in general are rarely described in the literature. We report the cytologic characteristics of heterotopic pancreatic tissue at the gastric outlet in a 48-year-old female. The patient underwent surgical excision due to symptoms related to the lesion. Endoscopic ultrasound fine-needle aspiration is increasingly used for the diagnosis of gastrointestinal tumors, which makes the recognition of certain endoscopically unreachable lesions an important step in optimal patient management.

Utility and diagnostic accuracy of endobronchial ultrasound-guided transbronchial fine-needle aspiration cytology of mediastinal lesions: Saudi Arabian experience.

OBJECTIVE: The objective of this study is to evaluate the cytological accuracy of endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-TFNA) of the mediastinal mass/nodular lesions. STUDY DESIGN: Over 3½ years from inception at King Khalid University Hospital, a retrospective analysis of the cytological diagnoses of all the EBUS-TFNA procedures performed in 80 patients who had mediastinal mass/nodular enlargement. Cytology results were reviewed and correlated with the histologic follow-up. RESULTS: Of the 80 patients who underwent EBUS-TFNA, 15 cases (18.75%) were positive for malignancy, 48 cases (60%) negative for malignancy and 17 cases (21.25%) unsatisfactory. Of the 48 cases, which were negative for malignancy, 24 (50%) cases were of granulomatous inflammation. The overall diagnostic yield of our EBUS-TFNA specimen was 78.75%. Forty-seven cases (58.75%) of 80 cases had histological follow-up biopsies. Among them, 32 cases (68%) had the same cytological and histological diagnosis and 15 cases (31.09%) had discordance between the cytology and the follow-up histological diagnosis. The sensitivity, specificity, and positive and negative predictive values for diagnosing granulomas by EBUS-TFNA are 77%, 82%, 83%, and 75% and for diagnosing malignancy are 71%, 100%, 100%, and 82%, respectively. CONCLUSION: Preliminary results show that cytological samples obtained through EBUS-TFNA are accurate and specific in making a diagnosis of the mediastinal mass/nodular lesions. Its optimum use depends on the effective collaboration between the cytotechnologist, pathologist, and the bronchoscopist.

Utility of endoscopic ultrasound-guided transbronchial fine-needle cytology in the diagnosis of sarcoidosis: A Saudi experience.

BACKGROUND: Endoscopic ultrasound-guided transbronchial fine-needle aspiration (EBUS-TFNA) is a minimally invasive technique for diagnosis of mediastinal masses/lesions. Although most studies have reported the utility of EBUS-TFNA in malignancy, its use has been extended to the benign conditions as well. OBJECTIVE: The present study focused on utility of EBUS in contributing to reach the final diagnosis of sarcoidosis. DESIGN: From May 2010 to December 2013, 19 of 80 patients who underwent EBUS-TFNA at one center for radiologically suspicious lesions for sarcoidosis, and with no definite histological diagnosis, were included in this retrospective study. RESULTS: When the histological diagnosis was taken as the gold standard, the sensitivity of EBUS-TFNA was 84.2% and specificity 100% with the positive predictive value of 100. The combined diagnostic sensitivity of EBUS-TFNA and transbronchial lung biopsy was 100%. CONCLUSION: EBUS is a valuable, minimally invasive diagnostic modality to support the diagnosis of sarcoidosis in appropriate clinical setting, after conventional work-up - particularly if patients have suspicious radiological findings. This minimally invasive procedure helps in providing a final diagnosis without exposing the patient to the risk of complications from more invasive procedures.

Menetrier`s disease in a Saudi child.

Menetrier`s disease is a rare form of acquired gastropathy that presents mostly during adulthood, but is extremely rare in children. It is a clinicopathological diagnosis that typically presents with abdominal pain, vomiting, and edema secondary to hypoalbuminemia. Endoscopy usually shows giant gastric mucosal folds, and gastric biopsy shows foveolar hyperplasia and decreased oxyntic glands. Here, we describe a 5-year-old boy from Saudi Arabia with typical presentation of Menetrier`s disease and serological evidence of acute cytomegalovirus infection.

Mimicry of sugar tumor and minute pulmonary meningothelial-like nodule to metastatic lung deposits in a patient with rectal adenocarcinoma.

Several reports have described different lung lesions mimicking primary or metastatic neoplasms. In this paper, we describe the different features of two uncommon and benign lung lesions mimicking metastasis from a primary large bowel adenocarcinoma. Our patient is a 75-year old female with a history of invasive rectal adenocarcinoma. One month after her surgery, she started complaining of coughing and shortness of breath. Clear cell sugar tumor and minute meningothelial-like nodules had been found incidentally and simultaneously during her chest x-ray. The diagnosis had been made based on morphology and was supported by a positive staining to a panel of immunohistochemical stains including CD34, vimentin, HMB45, melan A and S100. An ultra-structural examination was also performed and confirmed the presence of melanosomes in sugar tumor. The coexistence of lung sugar tumor and minute pulmonary meningothelial-like nodules has never been reported in the literature and an awareness of these lesions is essential to correctly diagnose and stage patients.

Abrupt withdrawal of inhaled corticosteroids does not result in spirometric deterioration in chronic obstructive pulmonary disease: Effect of phenotyping?

BACKGROUND AND OBJECTIVE: Some studies show a decline of FEV(1) only one month after withdrawal of inhaled corticosteroids (ICS), while others show no decline. We speculate that the presence of an asthma phenotype in the Chronic Obstructive Pulmonary Disease (COPD) population, and that its exclusion may result in no spirometric deterioration. METHODS: We performed a prospective clinical observation study on 32 patients who fulfilled the Global Initiative for Chronic Obstructive lung disease definition of COPD (Grade II-IV). They were divided into two phenotypic groups. 1. Irreversible asthma (A and B) (n = 13): A. Asthma: Bronchial biopsy shows diffuse thickening of basement membrane (≥ 6.6 µm). B. Airflow limitation (AFL) likely to be asthma: KCO > 80% predicted if the patient refused biopsy. 2. COPD (A and B) (n = 19): A. COPD: hypercapneic respiratory failure with raised bicarbonate, panlobular emphysema with multiple bullas, or bronchial biopsy showing squamous metaplasia and epithelial/subepithelial inflammation without thickening of the basement membrane. B. AFL likely to be COPD: KCO < 80% predicted. RESULTS: The asthma phenotype was significantly younger, had a strong association with hypertrophy of nasal turbinates, and registered a significant improvement of FEV(1) (350 ml) vs a decline of - 26.5 ml in the COPD phenotype following therapy with budesonide/formoterol for one year. Withdrawal of budesonide for 4 weeks in the COPD phenotype resulted in FEV(1) + 1.33% (SD ± 5.71) and FVC + 1.24% (SD ± 5.32); a change of <12% in all patients. CONCLUSIONS: We recorded no spirometric deterioration after exclusion of the asthma phenotype from a COPD group.

Comparison of three groups of patients with usual interstitial pneumonia.

OBJECTIVE: To determine the clinical characteristics and outcomes of patients with lungdominant connective tissue disease (LD-CTD) with a usual interstitial pneumonia (UIP) who do not meet the criteria for any form of CTD, and to compare these parameters with those of patients with idiopathic pulmonary fibrosis (IPF/UIP) and CTD-associated-UIP. METHODS: We conducted a prospective study on 118 patients diagnosed with UIP [LD-CTD, n = 28; CTDUIP, n = 29; and IPF/UIP, n = 61]. We compared the clinical characteristics, physiological findings, serum albumin concentrations, high-resolution computed tomography (HRCT) imaging data, and outcomes among the three groups and used Cox's proportional hazards regression analysis to identify variables associated with an increased risk of death. RESULTS: The LD-CTD and CTD-UIP patients were younger, more often female, and predominantly nonsmokers, compared with the IPF/UIP group. A significant difference in survival was evident between patients in the CTD-UIP and IPF/UIP groups (p = 0.028), but not between LD-CTD and IPF/UIP (p = 0.164) or between LD-CTD and CTD-UIP (p = 0.254). The variables associated with poorer survival in all UIP patients were (reduced) initial SpO2 level (hazard ratio [HR], 2.89; 95% confidence interval [CI] 2.1-3.7; p = 0.009) and lower serum albumin concentration (HR 2.16; 95% CI 1.6-2.7; p = 0.008). CONCLUSIONS: LD-CTD has distinct clinical characteristics that suggest an autoimmune background resembling that of CTD-UIP but differing from that of IPF/UIP. However, LD-CTD with a UIP pattern was not associated with improved survival. The resting oxygen saturation level and serum albumin concentration were independent predictors of mortality in all of the studied UIP patients, regardless of UIP type.

LPS-responsive beige-like anchor (LRBA) gene mutation in a family with inflammatory bowel disease and combined immunodeficiency.

BACKGROUND: Clinical immunology has traditionally relied on accurate phenotyping of the patient's immune dysfunction for the identification of a candidate gene or genes for sequencing and molecular confirmation. Although this is also true for other branches of medicine, the marked variability in immune-related phenotypes and the highly complex network of molecules that confer normal host immunity are challenges that clinical immunologists often face in their quest to establish a specific genetic diagnosis. OBJECTIVE: We sought to identify the underlying genetic cause in a consanguineous family with chronic inflammatory bowel disease-like disorder and combined immunodeficiency. METHODS: We performed exome sequencing followed by autozygome filtration. RESULTS: A truncating mutation in LPS-responsive beige-like anchor (LRBA), which abolished protein expression, was identified as the most likely candidate variant in this family. CONCLUSION: The combined exome sequencing and autozygosity mapping approach is a powerful tool in the study of atypical immune dysfunctions. We identify LRBA as a novel immunodeficiency candidate gene the precise role of which in the immune system requires future studies.

A 38-year old woman with a dural based lesion.

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare.We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38-year-old female. Xanthogranulomas are benign non-Langerhans cell histiocytic lesions. They are frequently described in the skin of infants and children. Extracutaneous manifestations especially in the central nervous system are highly uncommon. Dural xanthogranulomas usually arise in association with familial hypercholesterolemia, with Erdheim Chester disease (ECD), and with Weber-Christian disease. Our case however, had no such associations. In this report, the authors describe the clinical, radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma.

Fine needle aspiration cytology in the evaluation of lymphoid lesions: a retrospective study of the utility of flow cytometry in conjunction with morphology.

BACKGROUND AND OBJECTIVE: Fine needle aspiration (FNA) cytology, in conjunction with flow cytometry, is now widely used as a reliable and accurate method for the assessment of various lymphoid lesions, especially for lesions situated in odd locations where obtaining biopsy and monitoring for recurrence in previously diagnosed cases of lymphoma are difficult. The objective of this study was to determine the utility of FNA and immunophenotyping in the assessment of lymphoid lesions, and to find whether flow cytometry is more useful in the evaluation and subclassification of the small cell morphology group of lymphomas than in the large cell morphology group of lymphomas. DESIGN AND SETTING: Retrospective analysis of patients diagnosed with lymphoma over at a 5-year period. PATIENTS AND METHODS: All 175 FNA cases were followed carefully either clinically or histologically for at least 5 years. We compared the utility of flow cytometry in the diagnosis of small cell morphology lymphomas to large cell morphology lymphomas. RESULTS: Flow cytometry was performed on 72 of 175 (41%) of FNA specimens clinically suspicious of lymphoma. The excisional follow-up biopsy was obtained in 78 of 175 (44.5%) cases. Based on cytomorphologic evaluation, 82 cases (47%) were considered negative, 34 cases (19%) were considered atypical, 32 cases (18%) were positive for NHL-small cell morphology, 21 cases (12%) were positive for non-Hodgkin lymphoma (NHL)-large cell morphology, 3 cases (2%) were positive for NHL, and 3 cases (2%) were nondiagnostic. Immunophenotyping utilizing flow cytometry was the diagnostic parameter in 28 of 32 cases (88%) of the NHL-small cell morphology group and in 11 of 24 cases (46%) of the NHL-large cell morphology/Hodgkin lymphoma group. CONCLUSIONS: Immunophenotyping by flow cytometry is more essential for the accurate evaluation and classification of small cell morphology than large cell morphology lymphoid lesions in FNA cytology.

Low-grade malignant myoepithelioma arising in a pleomorphic adenoma: a rare case.

Malignant myoepithelioma is a very rare salivary gland tumor that can arise de novo or within a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma most probably arising in a pre-existing pleomorphic adenoma of the left parotid gland. The patient was a 60-year-old man who presented with a multinodular mass lesion over left side of the face and neck. He had undergone removal of a pleomorphic adenoma of the left parotid gland twice (8 and 22 years ago). Histological examination showed locally concentrated highly invasive myoepithelial cells with bland-looking morphology and no evidence of mitosis or necrosis. Immunohistochemistry confirmed the myoepithelial differentiation (S- 100+, SMA+) and a low Ki-67 labeling index (<5%).