First case report of acute myeloid sarcoma post renal transplant.

BACKGROUND: Although lymphoproliferative disorders are well-described and known entities post renal transplantation, acute myeloid sarcoma is a rare if ever reported occurrence in this setting. Immunosuppressive therapy is thought to be the main culprit in these cases. CASE REPORT: A 51-year-old man underwent renal transplantation 3.5 years earlier from a living unrelated donor. His posttransplant history was relevant for a road traffic accident after which a rise in the serum creatinine warranted 4 renal biopsies over a period of 7 months. The biopsies showed mainly acute tubular injury with sharp increase of the interstitial fibrosis and tubular atrophy between the first and the last biopsies. The patient was put on hemodialysis for 7 months, after which the allograft started functioning. The patient was followed a routinely, until he re-presented with increased creatinine and was found to have intra- and perirenal masses. Biopsies revealed acute myeloid sarcoma. The patient was started on fludarabine and cytosine arabinoside followed by All-trans retinoic acid and arsenic trioxide. CONCLUSIONS: The patient was disease free on his last positron emission tomography 1 year after the initial diagnosis.

Status of mismatch repair genes hMSH2 and hMSH6 in colorectal cancer in Saudi patients: an immunohistochemical analysis.

This study aimed to identify the status of 2 major microsatellite instability markers (repair genes hMSH2 and hMSH6) in colorectal cancer cases operated at King Khalid University Hospital, Riyadh, Saudi Arabia between 2007 and 2009. Immunohistochemical study of microsatellite instability was done with antibodies to hMSH2 and hMSH6. A total of 32 blocks were analysed from patients aged 16-83 years (median 56 years); 14 blocks (43.8%) were from resections and 18 (56.2%) were from biopsies. An adenomatous component was present in 4 (12.5%) blocks. The colonic carcinoma, the adenomas and the normal tissue showed strong nuclear reactivity to hMSH2 and hMSH6 in 96.9% of the cases. The rate of loss of expression was 3.1%. The rate of mutation in our sampled population was low and matched the rate reported in the literature from industrialized countries. Further studies are needed to confirm the use of these markers in the diagnosis of colorectal cancer.

Status of mismatch repair genes hMSH2 and hMSH6 in colorectal cancer in Saudi patients: an immunohistochemical analysis

This study aimed to identify the status of 2 major microsatellite instability markers [repair genes hMSH2and hMSH6] in colorectal cancer cases operated at King Khalid University Hospital, Riyadh, Saudi Arabia between 2007 and 2009. Immunohistochemical study of microsatellite instability was done with antibodies to hMSH2and hMSH6. A total of 32 blocks were analysed from patients aged 16-83 years [median 56 years]; 14 blocks [43.8%] were from resections and 18 [56.2%] were from biopsies. An adenomatous component was present in 4 [12.5%] blocks. The colonic carcinoma, the adenomas and the normal tissue showed strong nuclear reactivity to hMSH2and hMSH6in 96.9% of the cases. The rate of loss of expression was 3.1%. The rate of mutation in our sampled population was low and matched the rate reported in the literature from industrialized countries. Further studies are needed to confirm the use of these markers in the diagnosis of colorectal cancer

Nasopharyngeal T-cell monomorphic posttransplant lymphoproliferative disorders and combined IgA nephropathy and membranous glomerulonephritis in a patient with renal transplantation: a case report with literature review.

Recurrent and/or de novo glomerular diseases occurring in a renal allograft have been reported in the literature and are an important cause of graft dysfunction and eventual loss. The simultaneous occurrence of two glomerulonephritis, although reported in the literature, is a rare phenomenon. Posttransplant lymphoproliferative disorders (PTLD) are well known and one of the most serious and potentially fatal complications of chronic immunosuppression in the solid organ transplant recipient. Here, we are reporting the first case, to the best of our knowledge, of simultaneous occurrence of nasopharyngeal monomorphic monoclonal PTLD and two distinctive glomerular diseases (IgA nephropathy and membranous glomerulonephritis) in a 49-year-old patient who was 5 years post-renal transplantation. We have provided the clinical history of our patient who presented with nephrotic range proteinuria, microscopic hematuria, and a nasopharyngeal mass as well as a review of the literature.

Cytopathological grading, as a predictor of histopathological grade, in ductal carcinoma (NOS) of breast, on air-dried Diff-Quik smears.

Fine-needle aspiration cytology (FNAC) is a widely practiced technique in the diagnosis of breast carcinoma, and it is the only diagnostic procedure performed before definitive treatment, at most institutions. While the histological grading of breast carcinoma has become routine in many centers worldwide, the cytopathological grading of breast carcinoma is not commonly used. Grading of breast carcinoma, while the tumor is still in vivo, would be the most ideal and desirable situation, as it would be helpful in the selection of patients for appropriate therapy. The objective of this study, therefore, was to devise a simple system for grading breast carcinoma, based on the cytological features alone. We reviewed 125 cases of breast carcinoma retrospectively, which were initially diagnosed by FNAC, with subsequent histopathological confirmation. These included 105 ductal, 6 lobular, 2 tubular, 1 papillary, and 1 medullary carcinoma. There was 1 ductal carcinoma in situ. Nine cases were rendered insufficient for grading. Thus 105 cases of ductal carcinoma (NOS) were evaluated for final cytological grading. Air-dried Diff-Quik-stained smears were reviewed at least twice independently by four histopathologists and were then compared with the original histological grades. Six cytological features used for grading were found to be statistically significant: cellular pleomorphism, nuclear size, nuclear margin, nucleoli, naked tumor nuclei, and mitoses. A scoring system based on these six essential parameters was used, to classify ductal carcinoma into three cytological grades, which showed close correlation with the established histological grades. In addition, two less consistent, but still important, features were the presence or absence of necrosis and stromal invasion. Another six parameters, including smear cellularity, degree of cell dispersion or clustering, lymphoplasmacytic infiltrate, presence of tubular structures, cytoplasmic appearance of the tumor cells, and smear background, were not statistically significant. However, these additional parameters were found helpful in assigning the correct grade, in cases with borderline scores. The concordance rate with histology was 100% for grade 1, 98% for grade 2, and 93% for grade 3.

Paget's disease of the oesophagus associated with mucous gland carcinoma of the lower oesophagus.

AIM: To report a rare case of oesophageal Paget's disease and its rarer combination with submucosal gland carcinoma of the lower oesophagus. METHODS AND RESULTS: A 74-year-old Saudi female was admitted with the complaint of dysphagia. Endoscopic examination showed an ulcerated tumour at the gastro-oesophageal junction. Initial biopsy showed an undifferentiated carcinoma with pagetoid spread in the oesophageal stratified squamous epithelium. Oesophago-gastrectomy specimen showed a lobulated, poorly differentiated mucous gland carcinoma at the gastro-oesophageal junction. The tumour showed focal acinar differentiation and obvious cancerization of the submucosal glands, somewhat similar to the breast lobular carcinoma in situ. One of the isolated and cancerized submucosal glands also showed carcinoma in situ of its duct. Oesophageal surface epithelium showed extensive pagetoid spread, both over and away from the main tumour. The pagetoid tumour cells showed selective positivity for cytokeratin 7, cytokeratin Cam 5.2, BerEP4 and to a lesser extent for CEA. CONCLUSIONS: As far as we know, this is the fifth case report of oesophageal Paget's disease and the first report of its association with the underlying mucous gland carcinoma.

The value of CD34, myeloperoxidase and chloroacetate esterase (Leder) stain in the diagnosis of granulocytic sarcoma.

BACKGROUND: The differentiation of extramedullary myelogenous leukemia/granulocytic sarcoma (GS) from malignant lymphoma can sometimes be difficult. In the current study, we explored the value of CD34, myeloperoxidase and nonspecific esterase (Leder) stains in differentiating GS from lymphomas. MATERIALS AND METHODS: Fifteen cases of phenotypically confirmed GS were stained for CD34, myeloperoxidase and Leder stains. The same stains were performed in 16 malignant lymphomas as controls. The GS cases were also immunostained for CD3 and CD20 to detect the incidence of aberrant T and B lymphocyte expression. RESULTS: CD34 was expressed in 7 of the 15 cases of GS (46%). Myeloperoxidase was expressed in 10 of the 15 cases (66%), and Leder stain was positive in 9 cases (60%). All 15 cases had expression of at least one marker; 8 cases had expression of two markers and one case had expression of all 3 markers. None of the lymphomas showed expression of any of the three markers. Five cases (35%) of GS showed T cell antigen expression and 2 (14%) showed B cell antigen expression. CONCLUSION: Our findings suggest that in cases of GS, the use of the combination of CD34, myeloperoxidase and Leder stains can help reach a definitive diagnosis, especially if lymphoma is difficult to exclude. Expression of B and T cell antigens in such lesions should not rule out the diagnosis of GS.

Fine-needle aspiration biopsy of metastatic chordoma: case report and review of the literature.

Chordoma is a distinct malignant neoplasm arising from the remnants of the notochord and occurring mostly in the fifth or sixth decade of life, and occupying most frequently the sacral area (Bibbo, Comprehensive Cytopathology 1997; p 534). Metastases of the neoplasm may occur in 10-40% of cases (Jenkins et al., Clin. Radiol. 1995;50:416-417). Because of its rarity, the diagnosis of chordoma may be difficult to render, especially on fine-needle aspiration biopsy (FNAB). However, a clear-cut distinction of chordoma from other neoplasms is of utmost importance, since the prognosis and treatment of the patient will depend on the final diagnosis. This distinction in the case of metastases can be made easily, where correlation of previous histology has been done and/or ancillary studies have been performed. Diagn. Cytopathol. 2000;22:104-106.

Inflammatory myofibroblastic tumour of the tonsil.

Inflammatory myofibroblastic tumours are aetiologically enigmatic, nosologically confusing and biologically unpredictable lesions. The lungs are the organs of apparent predilection. These tumours have also been documented in a number of extrapulmonary sites including the head and neck. So far only two cases of inflammatory myofibroblastic tumour of the tonsil have been reported in the English literature. We document another case, occurring in a 41-year-old man with history of cadaveric renal transplant nine years ago. A comprehensive review of the literature is also presented.

B- and T-Lymphocyte distribution in benign and malignant lymphoepithelial lesions of the parotid gland: Correlation with Epstein-Barr virus expression and a proposed mechanism of malignant transformation.

Epstein-Barr virus expression in malignant lymphoepithelial lesions (LEL) of the parotid gland has been well established. The virus is occasionally expressed in benign LEL, especially in immunocompromised hosts. The pathogenesis of the disease as it relates to virus expression and lymphocyte subsets has not been clearly defined. In this study, we attempted to identify B- and T-lymphocyte distribution in the lesions as it relates to EBV expression in LELs of the parotid gland. Formalin-fixed paraffin-embedded sections of 18 cases of LEL of the parotid gland were immunohistochemically tested for the distribution of B- and T-lymphocytes in the lesions, using the antibodies L-26 (CD 20) for B-lymphocytes and UCHL-1 (CD-45RO) for T-lymphocytes. The sections were also tested by in situ hybridization for EBV mRNA expression, using the EBER-1 probe specific for EBV-1 gene. The 18 lesions included seven malignant LEL, seven benign LEL and four benign lymphoepithelial cysts. All malignant LELs showed a high and diffuse level of epithelial expression of EBV mRNA. Of the 11 benign lesions, only one case showed focal epithelial expression of EBV mRNA. This was a case of benign LEL in an HIV-positive male. All the benign lesions, except that expressing EBV mRNA, showed a T-/B-lymphocyte ratio averaging 2:1. All cases expressing EBV mRNA, including the case of benign LEL in the HIV-positive patient, showed a T-/B-lymphocyte ratio averaging 1:3. Our findings suggest that a T-lymphocyte-mediated immune response may play an essential role in suppressing proliferation of EBV in benign LEL of the parotid gland. This immune mechanism may be significantly disturbed in the malignant lesions, leading to uncontrolled viral replication and carcinogenesis.

Granulomatous lobular mastitis: A clinicopathological study of 112 cases.

We report 12 cases of granulomatous lobular mastitis occurring in young women, between ages 30 and 47 years, diagnosed at King Faisal Specialist Hospital and Research Centre (KFSH&RC) from 1987 to 1995. The disease was unilateral in 10 cases, while there was a history of involvement of the contralateral breast in two patients. At the time of diagnosis, two patients were lactating, two were pregnant, and two were pregnant and lactating. Histopathological examination in all cases revealed centrilobular granulomas and microabscess formation. Immunohistochemical staining in seven cases for T and B cell markers showed a predominance of T cells in the infiltrate in all cases. The treatment comprised surgical removal of the mass, debridement and antibiotics in some of the cases. In one patient referred from another institution, mastectomy had been performed on the basis of an erroneous histopathologic diagnosis of carcinoma. Preoperative diagnosis was carcinoma in seven cases and benign disease in the remaining five cases. Follow-up of the patients was uneventful in all cases. Granulomatous lobular mastitis is a rare inflammatory disease of the breast, which may clinically and pathologically mimic carcinoma, sometimes leading to misdiagnosis resulting in unnecessary surgery.

Hydatid cyst of the pterygopalatine-infratemporal fossa.

Hydatid disease is caused by the parasitic tapeworm. Echinococcus. This parasite in larval stage can thrive in many parts of the body, most commonly in the liver and the lung. Hydatid disease in the head and neck region is rare. An unusual location for hydatid disease in the pterygopalatine fossa-infratemporal fossa is presented. The patient did not have evidence of any other cyst on a ten-year follow-up.

Solitary fibrous tumor of the parapharyngeal space: a case report and review of the literature.

Solitary fibrous tumors are uncommon spindle cell neoplasms generally associated with serosal surfaces, especially the pleura. Recently, these tumors have been documented in a number of extrapleural sites including the head and neck. So far only two cases of parapharyngeal solitary fibrous tumor have been reported in the English literature. Rare location of an uncommon lesion often gives rise to difficulty in diagnosis or to misdiagnosis. In both the previously reported cases, as well as in our case, the diagnosis of solitary fibrous tumor was not made until the excised tumor was subjected to histopathology and immunohistochemistry.

Fine-needle aspiration biopsy diagnosis of extrarenal malignant rhabdoid tumor.

Two cases of extrarenal malignant rhabdoid tumors are presented in which diagnosis was suggested by fine-needle aspiration biopsy and confirmed by histologic and electron microscopic examination. Fine-needle aspiration smears in both cases revealed round to polygonal cells with vesicular nuclei and prominent nucleoli. Several tumor cells contained cytoplasmic inclusions composed of intermediate filaments. A majority of the tumor cells stained strongly for vimentin and cytokeratin. Electron microscopic examination revealed many cells with large aggregates of intermediate filaments corresponding to the cytoplasmic inclusions. Fine-needle aspiration biopsy may be used for diagnosing malignant rhabdoid tumor. The diagnosis may be further confirmed by immunohistochemistry and electron microscopy.