Extramedullary myeloid cell tumours localised to the mediastinum: a rare clinicopathological entity with unique karyotypic features.

Extramedullary myeloid cell tumour (EMMT) localised to the mediastinum is a rare manifestation of acute myeloid leukaemia, forming less than 4% of all cases of EMMT. In contrast to other types of EMMT, cytogenetic characteristics of this rare entity are relatively unknown. This report describes a patient with EMMT who had evidence of superior vena cava syndrome and normal peripheral blood counts at diagnosis. The results from an initial biopsy specimen were consistent with a diagnosis of mediastinal large B cell lymphoma. A diagnosis of acute myeloid leukaemia was made three months after initial diagnosis by bone marrow examination. Review of the initial biopsy specimen showed strong positivity for myeloperoxidase, revealing that the patient had been initially misdiagnosed as having large B cell lymphoma. Cytogenetic studies revealed a near triploid and near tetraploid karyotype with structural abnormalities in 12 and three metaphases, respectively. Review of the literature showed that a near tetraploid or triploid karyotype is found in most of the reported cases of mediastinal EMMT. Thus, the presence of a near triploid/tetraploid karyotype and mediastinal EMMT may represent a specific subset of EMMT. The biological relevance of this observation is discussed.

Unusual skin tumors in Langerhans' cell histiocytosis.

A case of Langerhans' cell histiocytosis with unusual skin manifestations in the form of multiple large skin tumors is described. The skin lesions responded partially to chemotherapy with etoposide and prednisone, and residual lesions were excised surgically. The patient developed central diabetes insipidus during treatment.

Spontaneous pneumothorax in metastatic choriocarcinoma.

We report a case of spontaneous bilateral pneumothoraces due to metastatic choriocarcinoma. The patient was successfully treated with tube thoracostomy and chemotherapy. Pneumothorax as a complication of choriocarcinoma has not been reported previously.

Cavitating pneumonia due to Trichosporon beigelii in a patient with acute myeloid leukaemia.

A 21-year-old man with acute myeloid leukaemia developed cavitating pneumonia while neutropenic and on broad spectrum antibiotics following induction chemotherapy. Trichosporon beigelii was isolated from several samples of sputum. He was successfully treated with amphotericin B. Previous reports of lung infection with this organism are reviewed.

Ceftazidime and amikacin as empiric treatment of febrile episodes in neutropenic patients in Saudi Arabia.

Sixty-four consecutive febrile episodes in 50 consecutive patients with malignancy and neutropenia were empirically treated with a combination of ceftazidime and amikacin. Of 52 analysable episodes, the response rate was 59.6% overall and 26.3% of episodes with microbiologically documented infections with septicaemia. Infection-related death occurred in 10 patients (19.2% of episodes). The response rates were similar in patients with acute leukaemia or other malignancies. Poor response is attributed to increased frequency of infections with Gram-positive and fungal organisms. A modified empiric regimen including cover for Gram-positive and fungal organisms is suggested in similar patient populations.

Correlation of fundus lesions and hematologic findings in leukemic retinopathy.

This prospective study evaluated the relationship between the fundus findings in leukemic retinopathy and hematologic parameters. Seventy-four newly diagnosed consecutive patients with acute leukemia were included, 49 with acute myelocytic leukemia (AML), and 25 acute lymphocytic leukemia (ALL). Blood parameters were based on data obtained before starting any therapeutic modalities. Leukemic retinopathy was detected in 32 patients (43%). Patients with ALL and retinal hemorrhages had significantly lower hemoglobin and hematocrit levels than those without hemorrhages (p = 0.004 and 0.018 respectively). AML patients with white-centered hemorrhages had a significantly higher leukocyte count than those without (p = 0.0002). ALL patients with cotton-wool spots had significantly lower hemoglobin levels and hematocrit than patients without such lesions (p = 0.044 and 0.05 respectively). AML patients with cotton wool spots had significantly lower leukocyte and platelet counts than those without (p = 0.019 and 0.003 respectively). Our results suggest that anemia is related to the findings of retinal hemorrhage and cotton-wool spots in ALL patients, that high leukocyte count is associated with white centered hemorrhage in AML patients, and that thrombocytopenia is not associated with retinal hemorrhage in this group of patients.

Primary gastrointestinal cancers in the Western Region of Saudi Arabia. Is the pattern changing?

OBJECTIVE: To determine the age, sex and relative frequencies of various gastrointestinal malignancies in patients registered with the National Cancer Registry from the Western Region of Saudi Arabia from January 1994 till December 1997, and compare this data with previous hospital based studies about the pattern of these malignancies in Saudi Arabia. METHODS: A National Cancer Registry was established in Saudi Arabia in 1992, and since 1st January 1994 all cancer cases in Saudi Arabia have been registered with the National Cancer Registry. All National Cancer Registry data on patients with primary gastrointestinal cancers from the Western Region of Saudi Arabia from January 1994 till December 1997 was retrieved and analyzed according to ethnic origin, site, age, sex and relative frequencies of various tumors. RESULTS: Out of a total of 1833 cases with primary gastrointestinal malignancies 1207 (66%) were Saudis, while the rest were Non-Saudis. Colorectal cancer was the the most common malignancy found in both population groups accounting for 28.5% of cases in Saudis and 36% in Non-Saudis. Malignancies of liver, stomach and esophagus followed in decreasing frequencies. The mean age of the Saudi population was 58+16 years (standard deviation) with male to female ratio of 1.67:1. About 80% of the patients were above 40 years of age and the peak of onset for most of the tumors was between 50 and 70 years of age. CONCLUSIONS: This study highlights that colorectal cancer is the most common gastrointestinal malignancy seen in the Western Region of Saudi Arabia followed by hepatocellular carcinoma. This is in sharp contrast to the previous hospital based studies from Saudi Arabia and national trends as seen in the cancer incidence report from the National Cancer Registry of Saudi Arabia. The factors for this changing pattern of gastrointestinal malignancy remain to the determined.

Pattern of primary gastrointestinal cancer: King Khalid University Hospital experience and review of published national data.

This study is based on data collected from a total of 629 patients (435 male and 194 female) with histoligically confirmed cancer of the primary gastrointestinal tract and its accessory glands, seen at King Khalid University Hospital (KKUH) between January 1985 and December 1993. During this period, a total of 2464 cancer cases were identified at KKUH. The relative frequency and rank of order are determined for various gastrointestinal cancers, as well as distribution according to age, sex and primary site affected. Gastrointestinal malignancy accounts for 25.5% of total cancer cases seen at KKUH. The mean age of patients was 59 +/- 17 years, with a male-to-female ratio 2.24:1. Primary liver cancer was the most common malignancy (34%), followed by gastric cancer (18%), colorectal (17%) and esophageal cancer (12%). We compared our finding with national published data from different provinces in Saudi Arabia. Results from this report stress the importance of prevention, screening and early detection for gastrointestinal malignancy.

Superior vena caval obstrution: King Khalid University Hospital experience.

A five year experience in 21 patients with superior vena caval obstruction (SVCO) was reviwed. SVCO was due to benign conditions in four patients (19%) and malignant tumors in 17 patients (81%). Lymphoma and lung cancer were the most common causes encountered. The most common symptoms and signs were facial swelling, shortness of breath, jugular venous distention, swelling of face and arms, and engorgement of thoracic veins. Benign disorders had a longer duration of symptoms before presentation and required longer time to make the diagnosis than in malignant disorders. No serious complications resulted from the superior vena cava obstruction itself or the investigate procedures leading to the diagnosis. Prognosis and response to treatment were dependent on the underlying cause of SVCO. Chemotherapy was effective in induction of complete regression of SVCO in the majority of patients with underlying malignant disorders. We conclude that SVCO should be approached invasively for diagnosis and tissue diagnosis of the underlying disorder should dictate the appropriate therapy.

Cancer at king khalid university hospital, riyadh.

This study is based on data collected from a total of 1196 patients (725 males and 471 females) with histologically confirmed cancer seen in King Khalid University Hospital over a 5-year period between September 1985 to August 1990. Four hundred forty-five patients were non-Saudi (37.20%). The relative frequency and rank of order are determined for various cancers in Saudi patients and total group and compared with the results of the other published studies on cancer epidemiology in Saudi Arabia. The most common cancers among Saudi males were liver, non-Hodgkin's lymphomas, stomach, lung, central nervous system, prostate, lymphoid, leukemias, myeloid leukemias, urinary bladder and Hodgkin's lymphomas, central nervous cancers among Saudi females were breast, thyroid, non-Hodgkin's lymphomas, central nervous system, stomach, myeloid leukemias, esophagus, lymphoid leukemias, liver and ovary. Rank order and relative frequency for liver cancer in both sexes is the highest among any study previously published on the epidemiology of cancer in Saudi Arabia.

Pernicious anemia in Saudi Arabs.

Over a five-year period extending from January 1986 to December 1990, seven cases of pernicious anemia in Saudi patients were diagnosed at King Khalid University Hospital in Riyadh. There were five males and two females. The age range was 45 to 73 with a mean age of 61 years. The presenting symptoms, laboratory features and the disease pattern were similar to those described in northern European patients with the possible exception of male predominance in our patients. One patient demonstrated an interesting phenomenon of masking the macrocytosis of pernicious anemia by concurrent beta thalassemia minor. No association with any other autoimmune diseases was detected in any of our patients.

Adult acute myeloblastic leukemia: Experience at King Khalid University Hospital.

BACKGROUND: The clinical features of acute myeloblastic leukemia (AML) and its response to therapy in adult patients in Saudi Arabia are not well defined, as only scanty data has been available. This situation will likely continue unless experience with AML is reported from different institutions in the Kingdom. PATIENTS AND METHODS: In this retrospective study, the records of 52 adult patients with previously untreated de novo acute myeloblastic leukemia (AML) who were treated at King Khalid University Hospital over a five-year period from January 1989 to December 1993 according to the conventional â3+7â regimen were reviewed. The clinical features of the disease, response to therapy and treatment-related complications were identified. RESULTS: There were 33 males and 19 females with a mean age of 30+/-13 years (mean+/-SD). M 4 and M 5 AML were the predominant French-American-British (FAB) subtypes encountered. Sixty-five percent of patients achieved complete remission (CR). The median duration of the first CR of all analyzable patients was 32 weeks. The median CR duration and survival of patients achieving complete remission who survived through their consolidation treatment was 36 and 49 weeks, respectively. CONCLUSION: Both median duration of the first complete remission and survival compare unfavorably with those reported in the literature despite a comparable remission rate. Infectious complications were frequent and accounted for a significant number of mortalities.

Primary non-Hodgkin's lymphoma of liver with humoral hypercalcaemia.

A case of primary non-Hodgkin's lymphoma of the liver with associated humoral hypercalcaemia is described. The patient was successfully treated with combination chemotherapy. A similar association has not been reported previously.

Primary gastric non-Hodgkin's lymphoma: clinical features, management, and prognosis of 185 patients with diffuse large B-cell lymphoma.

BACKGROUND: Primary gastric non-Hodgkin's lymphoma (PG-NHL) is common in Saudi Arabia. This has prompted the analysis of a large series of patients with PG-NHL having high-grade diffuse large B-cell lymphoma (DLCL) in order to define the clinical features and outcome of this disease. PATIENTS AND METHODS: The data of all adult patients in the series with PG-NHL having DLCL histology were retrospectively reviewed. Patients were eligible if they had biopsy-confirmed diagnoses obtained by endoscopy or following laparotomy. RESULTS: Over a 16-year period, 185 patients with DLCL PG-NHL were identified and their data were reviewed. Patients had a median age of 54 years. In 53% of them only one initial therapeutic modality was given, while 47% were managed by a multi-modality approach. One hundred forty patients (76%), 19 (10%), and 26 (14%) attained complete remission (CR), partial remission, and no response/progressive disease, respectively. Multivariate analysis showed that poor performance status and advanced stage were negatively associated with the likelihood of attaining CR. Over a median follow-up of 54 months, 118 (64%) of the patients were alive and disease-free, 17 (9%) were alive with evidence of disease, and the remaining 50 (27%) were dead. The projected 5-year and 10-year overall survivals (OS) (+/- SD) were 68% (+/- 4%) and 61% (+/- 6%), respectively. The Cox proportional hazards model identified the same variables of response as adverse prognostic factors of survival. Using the influence of performance status, and stage, a prognostic index was constructed to recognize three prognostically distinctive risk categories with overall survival proportions of 87%, 61%, and 45%, respectively. The unadjusted International Prognostic Index, however, failed to classify patients into prognostically meaningful risk strata. Of the 140 patients who achieved CR, the median disease-free survival (DFS) was not reached, but the predicted 5- and 10-year DFS were 82% and 75%, respectively. A multivariate analysis identified poor performance status as the only independent prognostic covariate that adversely influenced DFS. Our analysis showed that compared with single-modality management, multi-modality strategy attained significantly higher CR, and advantageous OS and DFS. CONCLUSIONS: This large series characterized the clinico-pathologic features and outcome of patients with DLCL PG-NHL. Performance status, and stage significantly influenced patient outcome. A prognostic index was developed and it identified three prognostically distinctive risk groups; however, prospective validation is warranted.

High complete pathological response in locally advanced breast cancer using paclitaxel and cisplatin.

BACKGROUND: In an earlier study, we have demonstrated a high response rate in metastatic breast cancer using paclitaxel (P) and cisplatin (C). A phase II study using the same regimen (PC) has been conducted in locally advanced breast cancer (LABC). METHODS: A total of 72 consecutive patients with non-inflammatory LABC (T2 > or = 4 cm, T3 or T4, N0-N2, M0). Patients were scheduled to receive 3-4 cycles of the neoadjuvant PC (paclitaxel 135 mg/m2 and cisplatin 75 mg/m2 on day 1) every 21 days. Patients were then subjected to surgery and subsequently received 6 cycles of FAC (5-fluorouracil 500 mg/m2, doxorubicin 50 mg/m2, and cyclophosphamide 500 mg/m2) or 4 cycles of AC (doxorubicin 60 mg/m2, and cyclophosphamide 600 mg/m2). Patients then received radiation therapy, and those with hormone receptor positive tumors were given adjuvant tamoxifen intended for 5 years. RESULTS: The median age was 39 years (range, 24-78). Clinically, 7%, 58%, and 35% of patients had T2 > or = 4 cm, T3, and T4, respectively. Disease stage at diagnosis was IIB (33%), IIIA (27%), and IIIB (40%). Complete and partial clinical response to PC was demonstrated in 13 (18%), and 52 (72%) patients, respectively. Of those patients with evaluable pathologic response (68 patients), complete pathologic response (pCR) was achieved in 15 (22%) patients. At a median follow-up of 22 (+/- 3.5) months, 58 (81%) were alive with no recurrence, nine (12%) were alive with evidence of disease, and five (7%) were dead. None of the patients achieving pCR has developed any relapse. The median overall survival has not been reached for all 72 patients with a projected 3-year survival (+/- SE) of 90% (+/- 4%). The median progression-free survival (PFS) was 42.1 (+/- 4.8) months with a projected PFS of 74% +/- 7% at 3-years (for 68 patients). CONCLUSIONS: PC regimen in LABC produced a high pCR. The contribution of the other added modalities to survival could not be assessed.

Granulocyte-Macrophage Colony-Stimulating Factor in Newly Diagnosed Adult Patients with Acute Lymphoblastic Leukemia Treated with Conventional Chemotherapy.

Twenty consecutive adult patients with newly diagnosed acute lymphoblastic leukemia (ALL) were treated with conventional therapy consisting of daunorubicin, vincristine, prednisone and L-asparaginase in standard doses. Granulocyte-macrophage colony-stimulating factor (GM-CSF) was administered at a single subcutaneous daily dose of 5 microgram per kilogram body weight for fourteen days starting on day 7 of chemotherapy. Twenty two adult patients with acute lymphoblastic leukemia and similar risk characteristics who received the same chemotherapeutic regimen without GM-CSF served as a historical control group. The complete remission rate and the rate of early mortality were similar in both groups of patients. Patients treated with GM-CSF showed significantly faster neutrophil recovery above 0.5 × 10(9)/L than the control patients (P < 0.005). The incidence of febrile episodes and the rate of documented infection were similar in the two groups of patients.