Symptomatic Giant Cavernous Haemangioma of the Liver: Is Enucleation a Safe Method?

Twenty-three patients with symptomatic giant hemangioma of the liver were treated by surgery between 1979 and 1996 at the department of General Surgery, Faculty of Medicine, University of Çukurova. Twenty-three enucleations were performed in 21 patients, left lateral segmentectomy in one patient and enucleation plus left lobectomy in one patient. The tumors were enucleated along the interface between the hemangioma and normal liver tissue. The diameters of the tumors ranged from 5×5 to 25×15 cm. The mean blood loss for enucleations was 525 ml (range 500–1000 ml). There was no mortality and no postoperative bleeding. Three patients had postoperative complications. Enucleation is the best surgical technique for symptomatic giant hemangioma of the liver. It may be performed with no mortality, low morbidity and the preservation of all normal liver parenchyma

Leukaemia cutis and leukaemic vasculitis

PubMedID: 11069455Background: Leukaemia cutis (LC) and leukaemic vasculitis (LV) are uncommon but important features of haematological malignancy. Objectives: We aimed to evaluate clinicopathological features of a series of such cases. Methods: Thirty-four patients with LC were studied. Nineteen had acute myeloblastic leukaemia, six had acute lymphoblastic leukaemia, three had myelodysplastic syndrome-refractory anaemia with excess blast in transformation, three had non-Hodgkin's lymphoma in leukaemic phase, two had chronic lymphocytic leukaemia and one had multiple myeloma. Results: Cutaneous lesions were widely dispersed in 15 patients, but were restricted to the extremities in eight patients. In other patients lesions were localized on the face, hands and feet. Cutaneous lesions were generally erythematous, maculopapular, squamous, nodular, haemorrhagic or necrotic. Two localizations were very interesting: in one case, lesions were confined to the site of previous varicella zoster virus infection; in the other case, leukaemic infiltration occurred within a lesion of cutaneous leishmaniasis. All of the patients had high white blood cell count and/or other findings of high tumour burden. Vasculitis with leukaemic cell infiltration (LV) was detected in 24 patients, and was high-grade LV in 16 patients. Patients with high-grade LV tended to have a higher white blood cell count and other findings associated with an aggressive clinical course compared with patients not having LV. Thirteen patients with LV died within 1 month but five patients lived more than 1 year. Conclusions: LC, and especially LV, is generally an indicator of poor prognosis. Its pathogenesis must be evaluated further with additional studies, including adhesion molecules, angiogenic factors and other biological parameters

BOTRYOID NEPHROBLASTOMA (WILMS-TUMOR) IN A HYPOPLASTIC KIDNEY

WOS: A1987L332700005PubMed ID: 2829401

Human Herpes virus-8 related Kaposi Sarcoma of the ureter after renal transplantation

WOS: 000388614502611

Giant axonal neuropathy in two siblings: Clinical histopathological findings

PubMedID: 15696785Objective: Giant axonal neuropathy is a rare, severe autosomal-recessive neurologic disease affecting both the peripheral and the central nervous system. In this article, we describe a detailed clinicopathological report of two affected sibs from a consanguineous Turkish family. Patients: The index patient was a 6.5-year-old girl. Her intellectual development was normal. At the age of 3, her parents noticed progressive lack of balance and deterioration of motor skills. On examination, she had paresis and sensory loss more marked distally. Her mental status was normal. Her older brother had similar findings. Results: Electrophysiological studies of young patients showed decreased median and ulnar nerve conduction velocities, absent peroneal motor potential, absent sensory nerve potentials and an EMG suggesting a neurogenic pattern. MRI showed mild cerebral and cerebellar atrophy. The nerve biopsy showed moderate myelinated nerve fibres loss, several regenerative clusters and multiple giant axons. Focal demyelination, hypertrophic "onion pulp" changes and endoneural fibrosis were also seen. Immunohistochemically, neurofilament protein accumulation was detectable in giant axons. Conclusions: This consanguineous family with two affected siblings and healthy parents complies with autosomal-recessive inheritance in GAN. In the majority of reported GAN cases, CNS involvement is described early in the course of the disease, but these patients did not present any sign of CNS involvement. GAN is a rare genetic disease of childhood involving the central and peripheral nervous systems. The diagnosis is easy with clinical, electrophysiological, and histopathological features, if it has been done. Early diagnosis is important, because of possible prenatal diagnosis

Vasculitis and leukemia

PubMedID: 11426610Vasculitis may accompany neoplasias and be of paraneoplastic type or associated with drugs used in patient treatment. We evaluated skin biopsies of twenty-eight cases with vasculitis accompanying leukemias reviewed and clinical outcome was evaluated. Eleven of the 28 cases had paraneoplastic vasculitis and 17 had vasculitis associated with various drugs including chemotherapy, cytokines and antibacterial agents. Paraneoplastic vasculitis was seen in 3 cases with chronic myelocytic leukemia in blastic phase, 5 patients with acute myeloblastic leukemia, and 3 with myelodysplastic syndrome. Drugs responsible for the 17 cases of vasculitis included hydroxyurea, vincristine, cytosine-arabinoside, methotrexate, all-trans retinoic acid, granulocyte-colony stimulating factor, interferon and antibiotics. Paraneoplastic vasculitis is not rare in leukemias and may be a manifestation of the blastic phase of chronic myeloid leukemia. Furthermore paraneoplastic vasculitis can be fatal in myelodysplastic syndromes and may be present clinically before the specific diagnosis is made. Drugs used in routine therapy may be the cause of the vasculitis, thus skin biopsy should be performed in all cutaneous lesions in patients with hemopoietic neoplasias

Granulocytic sarcoma: 32 cases and review of the literature

PubMedID: 17169797Thirty-two cases of granulocytic sarcoma (GS) are reported in this paper. Age range was from 16-70 years. GS was accompanied by AML in 13 cases, ALL (My+) in one case, CML in 11 cases and MDS in two cases. GS was diagnosed simultaneously with leukemia in five cases and preceded the leukemia in eight. Lymph node and soft tissue were the most commonly detected localizations. Seven cases had first been diagnosed as NHL. Histopathologically blastic, immature and mature variants were found in 11, nine and 11 cases respectively and overall survival was shortest in the blastic type. Myeloperoxidase and lysozyme were found to be positive in 30 and 24 cases respectively. Therapy was radiation in five cases and surgery in three. Systemic chemotherapy was given to the cases. The clinical outcome of the patients after the diagnosis of GS was poor. GS is a unique entity; prognosis is poor but it is important to detect the signaling pathways associated with migration of myeloid cells to the extra-medullary tissues. The critical factors for detecting this interesting tumor are to be aware of this disease, cooperation between clinician and pathologist and the application of special stains to detect the myeloid origin

PYLORIC ATRESIA - A CASE-REPORT

WOS: A1988AD05100007PubMed ID: 3242184

Splenic involvement in a stillborn fetus with tuberous sclerosis and multiple cardiac rhabdomyomas

PubMedID: 15641272Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis (TSC). However, splenic involvement in TSC is very rare. Histiocytoid cells in the spleen have been previously reported in only seven neonates and one fetus. We report an unusual case of multiple cardiac rhabdomyomas in a stillborn fetus with TSC who had clusters of histiocytoid cells in the spleen. These large cells had abundant eosinophilic cytoplasm and were positive with CD 68; ultrastructurally, they contained many membrane-bound bodies. It has been suggested that these cells are histiocytes. To the best of our knowledge, the present case is the second stillborn fetus who had splenic involvement with TSC