Gastrointestinal Stromal Tumor: A Rare Abdominal Tumor

Gastrointestinal stromal tumors (GISTs) are rare abdominal tumors which arise from the interstitial cells of Cajal in the gastrointestinal tract. Gastric GISTs are the most commonly seen GIST tumors and may grow to a very large size. They are often associated with abdominal pain, anorexia and weight loss. Most of them can be detected by CT. These tumors have been found to harbor mutations in CD117 which causes constitutional activation of the tyrosine kinase signaling pathway and is considered to be pathognomic. Tyrosine kinase inhibitors such as imatinib have revolutionized the treatment of these tumors, which are otherwise resistant to conventional chemotherapy and radiotherapy. Although surgical resection is the mainstay of treatment, tyrosine kinase inhibitors have been useful in prolonging the recurrence-free survival of these patients. Resistance to imatinib has been reported in GISTs with specific mutations. We present a case of gastric GIST which grew to a very large size and was associated with abdominal pain and weight loss. It was successfully resected and the patient was commenced on imatinib therapy

Pulmonary Lymphangitic Carcinomatosis due to Renal Cell Carcinoma

Renal cell carcinoma is an aggressive disease with a high rate of mortality. It is known to metastasize to the lung, liver, bone and brain. However, manifestation through lymphatic spread to the lungs is rare. Lymphangitic carcinomatosis is commonly observed in malignancies of the breast, lung, pancreas, colon and cervix. It is unusual to observe lymphangitic carcinomatosis of the lungs due to renal cell carcinoma. Lymphangitic carcinomatosis of the lungs may result in severe respiratory distress and may be the direct cause of death. Currently, there are no known modalities of preventing or slowing lymphangitic carcinomatosis besides treating the primary tumor. However, early detection may change the course of the disease and may prolong survival. This is compounded by the difficulty involved in diagnosing lymphangitic carcinomatosis of the lung which frequently involves lung biopsy. Immunohistochemical studies are often used in conjunction with regular histochemistry in ascertaining the primary tumor and in differentiating it from pulmonary metastasis. In this case report, we describe the presentation and clinical course of renal cell carcinoma in a patient which manifested as lymphangitis carcinomatosa of the lungs. The patient underwent surgical resection of the primary tumor with lymph node resection but presented with a fulminant lymphangitic carcinomatosis of the lungs within two weeks. Immunohistochemistry of the tissue obtained by the biopsy confirmed the diagnosis which was subsequently corroborated during his autopsy. This case illustrates the necessity of an urgent follow-up of chemotherapy and immunotherapy in such patients

Benign Metastatic Leiomyoma Presenting as a Hemothorax

Uterine leiomyomas have been reported to metastasize to various organs including the lungs, skeletal muscles, bone marrow, peritoneum, and heart. They may present with symptoms related to the metastases several years after hysterectomy. These tumors regress after menopause, and it is rare to detect active tumors in postmenopausal women. Despite their ability to metastasize, they are considered to be benign due to the lack of anaplasia. Pulmonary benign metastasizing leiomyoma is usually detected in the form of pulmonary nodules incidentally on imaging. Tissue biopsy of these nodules is required to identify them as benign metastasizing leiomyomas. Immunohistochemical analysis and molecular profiling may further help detect any malignant transformation in it. Untreated pulmonary benign metastasizing leiomyoma may result in the formation of cystic structures, destruction of lung parenchyma, and hemothorax and may cause respiratory failure. Surgical resection and hormonal therapy help prevent progression of this disease and provide an avenue for a cure

Giant Adrenal Myelolipoma Masquerading as Heart Failure

Adrenal myelolipomas are rare benign tumors of the adrenal cortex composed of adipose and hematopoietic cells. They have been postulated to arise from repeated stimulation by stress, inflammation and ACTH oversecretion. Myelolipomas are usually detected incidentally on imaging and do not require any active intervention besides regular follow-up by imaging. However, myelolipomas may insidiously grow to large sizes and cause mass effects and hemorrhage. Timely diagnosis and surgical resection are curative and lifesaving

Diffuse Alveolar Hemorrhage due to Acute Mitral Valve Regurgitation

Diffuse alveolar hemorrhage (DAH) can be caused by several etiologies including vasculitis, drug exposure, anticoagulants, infections, mitral valve stenosis, and regurgitation. Chronic mitral valve regurgitation (MR) has been well documented as an etiological factor for DAH, but there have been only a few cases which have reported acute mitral valve regurgitation as an etiology of DAH. Acute mitral valve regurgitation can be a life-threatening condition and often requires urgent intervention. In rare cases, acute mitral regurgitation may result in a regurgitant jet which is directed towards the right upper pulmonary vein and may specifically cause right-sided pulmonary edema and right-sided DAH. Surgical repair of the mitral valve results in rapid resolution of DAH. Acute MR should be considered as a possible etiology in patients presenting with unilateral pulmonary edema, hemoptysis, and DAH

Extracavitary Manifestation of Primary Effusion Lymphoma as a Right Atrial Mass

Primary effusion lymphoma (PEL) is a subset of large B cell lymphomas and has been mostly associated with human immunodeficiency virus infection. Rare cases have been reported in organ transplant recipients and chronic hepatitis C patients. It typically presents as an effusion in the pleural and pericardial spaces but rarely disseminates. However, involvement of the gastrointestinal tract, lymph nodes and bone marrow has been reported. Diagnosis is based on characteristic clinical, histopathological and immunohistochemical features. We present a case with a right atrial mass which tested positive for human herpes virus 8 (HHV-8), CD20, CD30 and lambda light chains and negative for CD138, kappa light chain, PAX5, Epstein-Barr virus, latent membrane protein 1, CD2, CD3, CD8 and CD56. Bilateral pleural effusions and pericardial effusions were noted which tested positive for HHV-8, CD30 and CD45. The patient responded well to the R-EPOCH regimen with complete resolution of the effusions and a significant decrease in the size of the right atrial mass. This case report illustrates the atypical manifestation of PEL as a right atrial mass

Uterine Leiomyosarcoma Manifesting as a Tricuspid Valve Mass

Uterine leiomyosarcoma is a rare malignancy and carries a poorer prognosis when compared to endometrial carcinoma. It has been observed to metastasize to all the major organs. It presents with symptoms of abdominal distension, vaginal bleeding and may pass unnoticed until an advanced stage in patients with leiomyomas. Surgery is a viable option in patients with disease limited to the uterus, but metastasis to the heart may require surgery to prevent acute and catastrophic complications. The case described here involves metastasis to the tricuspid valve, which caused severe tricuspid regurgitation in the setting of acute pulmonary embolism. Surgical resection restored cardiac function and stabilized the patient. This case illustrates a rare site of metastasis of leiomyosarcoma which required immediate intervention and resulted in a favorable outcome

Ovarian Hyperstimulation Syndrome as an Etiology of Obstructive Uropathy

Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic complication of controlled ovarian hyperstimulation (COH) protocols performed in women undergoing assisted reproductive technologies. Overstimulation of the ovaries results in the overproduction of vasoactive cytokines and mediators by the ovaries, thereby causing a generalized capillary leak and acute shift of protein-rich fluid from the intravascular compartment into the third space. This may lead to the development of ascites, pleural effusions, pericardial effusion, anasarca, intravascular volume depletion, hemoconcentration, oliguria, hypoalbuminemia and hypoproteinemia, electrolyte imbalances, acute renal failure, abdominal compartment syndrome, thromboembolic events, and adult respiratory distress syndrome. The only effective treatment available is prevention of the syndrome from developing by individualizing the stimulation protocol, especially in high-risk patients. Once the syndrome develops, the management is mainly supportive. Oliguria and some degree of acute renal failure commonly develop in patients with moderate to severe OHSS and are usually due to prerenal causes. Acute renal failure (ARF) secondary to obstructive uropathy is rare. Here we report a case of severe, life-threatening OHSS resulting in ARF secondary to obstructive uropathy

Renal Medullary Cancer in a Patient with Sickle Cell Trait

Renal medullary cancer is a rare malignancy almost exclusively seen in young patients of African ethnicity. These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait. It is estimated that 300 million people worldwide carry sickle cell trait, and the presence of hematuria in these patients should be treated as a harbinger of a possible malignancy. Notably, this tumor mostly develops on the right side of the body. Patients often present with it at an advanced stage and the prognosis is poor. Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis. Current chemotherapy options are very limited, and early detection may provide a chance for surgical resection. It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens

Exercise capacity as an independent risk factor for adverse cardiovascular outcomes among nondiabetic and diabetic patients

Introduction: To investigate if decreased exercise capacity is an independent risk factor for major adverse cardiovascular events (MACE) in diabetics and nondiabetics. Material and methods The association of decreased exercise capacity (EC) during a treadmill exercise sestamibi stress test with MACE was investigated in 490 nondiabetics and 404 diabetics. Mean follow-up was 53 months. Results: Nondiabetics with a predicted EC < 85% had a higher prevalence of myocardial ischemia (34% vs. 19%, p = 0.0002), 2- or 3-vessel obstructive coronary artery disease (CAD) (31% vs. 13%, p = 0.016), myocardial infarction (MI) (17% vs. 7%, p = 0.0005), stroke (8% vs. 2%, p = 0.002), death (11% vs. 3%, p = 0.0002), and MI or stroke or death at follow-up (32% vs. 11%, p < 0.001) compared to nondiabetics with a predicted EC ≥ 85%. Diabetics with a predicted EC < 85% had a higher prevalence of myocardial ischemia (48% vs. 32%, p = 0.0009), 2- or 3-vessel obstructive CAD (54% vs. 28%, p = 0.001), MI (32% vs. 14%, p < 0.001), stroke (22% vs. 6%, p < 0.001), death (17% vs. 9%, p = 0.031), and MI or stroke or death at follow-up (65% vs. 27%, p < 0.001). Stepwise Cox regression analysis showed decreased EC was an independent and significant risk factor for MACE among nondiabetics (hazard ratio 3.3, p < 0.0001) and diabetics (hazard ratio 2.7, p < 0.0001). Conclusions: Diabetics and nondiabetics with decreased EC were at increased risk for MACE with nondiabetics and decreased EC at similar risk as diabetics with normal EC