1/11. Recurrent adrenocortical carcinoma in a child.An 8-month-old girl presented with clitoromegaly, cushingoid features and a large abdominal tumour. ultrasonography (US) and computed tomography (CT) of the abdomen revealed a tumour of the left suprarenal gland, 12x11x7 cm in size. serum levels of cortisol, testosterone and DHEA-S, and urinary extretion of 17-ketosteroids and 17-hydroxycorticoids were increased. Complete removal of the tumour was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically. Three months after the first operation, a recurrent tumour of the left renal hilus, 23x15 mm in size, was identified by US and verified by aspiration biopsy. The tumour was removed by the transabdominal route. In this report, we discuss the diagnosis and the treatment of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/11. Angiotropic lymphoma manifesting as a solitary adrenal tumor in a case of ankylosing spondylitis.Angiotropic lymphoma is an extremely rare disease characterized by intravascular accumulation of large neoplastic lymphocytes, with the clinical manifestations of fever, skin lesions and neurologic deficits. We report a patient who developed angiotropic lymphoma after a 10-year history of ankylosing spondylitis. The clinical disease manifested as a unilateral, solitary adrenal tumor, fever and body weight loss. The fever subsided after surgical removal of the adrenal tumor. Systemic chemotherapy was administered postoperatively. The patient was leading an uneventful life 44 months after the initial diagnosis. To our knowledge, this is the first case of angiotropic lymphoma associated with ankylosing spondylitis. It is also the second reported case manifesting with a unilateral solitary adrenal tumor without systemic involvement.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/11. A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement.Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/11. Phaeochromocytoma in children.Phaeochromocytoma is a rare disease in childhood with a subtle and wide range of clinical presentations. We report two confirmed cases and one potential case of phaeochromocytoma, each belonging to a different disease spectrum or syndromal disorder, namely sporadic phaeochromocytoma, von hippel-lindau disease, and multiple endocrine neoplasia type 2a. knowledge of the molecular basis of the condition helps to make the diagnosis. Affected individuals and their family members should be screened for any associated syndromal disorders that can carry a substantial degree of morbidity and mortality.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/11. Adrenal metastatic placental site trophoblastic tumor. Case report.Placental site trophoblastic tumor is a rare disease. Since its first description in the literature only about 100 cases have been reported. Initially considered a benign pathology, its malignant potential with metastases was later recognized. About 30% of the cases described metastatised, thereby aggravating the prognosis. The therapy is surgical and in cases of metastatised disease has to be supplemented by chemotherapy. A case of placental site trophoblastic tumour with a single metastasis to the left adrenal gland treated with adrenalectomy and chemotherapy is reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/11. Bilateral pheochromocytoma during pregnancy.BACKGROUND: pheochromocytoma is a rare disease of the chromaffin cells that secrete catecholamines. It may occur during pregnancy. Bilateral pheochromocytoma in pregnancy is even rarer. CASE: A 26-year-old woman, gravida 2, para 0-0-1-0, 18 weeks' pregnancy, was initially seen with elevated blood pressure (170/100 mmHg) and mild headache. The cause of hypertension was conventionally investigated and bilateral pheochromocytoma was finally searched for and found. Bilateral adrenalectomy was undertaken at 23 weeks' gestation and cesarean section was performed at 31 weeks' gestation due to intrauterine growth retardation (IUGR) and compromised fetal well-being. The maternal outcome was uneventful and the baby was physiologically complicated only by neonatal jaundice. CONCLUSION: pheochromocytoma should be searched for in the conventionally differential diagnosis in hypertension during pregnancy, especially in the young. early diagnosis and proper management with medical treatment followed by surgical removal of the tumor usually result in good maternal and fetal outcomes.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/11. Symptomatic adrenal myelolipoma: therapeutic considerations.Four cases of symptomatic myelolipoma of the adrenal gland are presented. Three were treated at laparotomy and one with a laparoscopic approach. On the basis of these four cases we discuss the aetiology, pathogenesis, clinical manifestations, diagnosis and treatment of this rare disease. We also report the follow-up of a patient with bilateral myelolipoma, who underwent monolateral excision. We conclude that myelolipoma, if correctly diagnosed, can be treated conservatively with careful follow-up, limiting surgery to symptomatic cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/11. pheochromocytoma in pregnancy: case report and review of the literature.pheochromocytoma is a rare disease that may occur during pregnancy. Only a few hundred cases have been published in the literature. Manifestations include hypertension with various clinical presentations, possibly resembling those of pregnancy-induced hypertension, or pre-eclamptic toxemia. Differentiation of these conditions is not always feasible, thus creating a serious risk, because fetal and maternal morbidity and mortality are far higher with pheochromocytoma. Biochemical measurements of catecholamines and their metabolites are apparently a convenient way to establish diagnosis during pregnancy, inasmuch as interpretation of radiological evaluation is complicated by the gravid uterus, and might even be potentially dangerous due to the use of ionizing radiation. More sophisticated methods for evaluation are not always practical during pregnancy. Medical treatment aims at controlling symptoms, mandating the use of alpha- and beta-receptors blockade medication. Surgical intervention is the only possible curative method available, but the critical issue is probably to identify the exact timing during the course of pregnancy for such intervention, or the ability to control symptoms until delivery. Although malignant transformation of pheochromocytoma have been reported, it is extremely uncommon. The overall prognosis is mainly affected by early diagnosis, and multidisciplinarian management.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/11. Cystic adrenal mass: pseudocyst associated with hemangioma.We present a 70-year-old man with cystic adrenal tumor associated with hemangioma. The pathogenesis of this rare disease is discussed with special reference to its diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/11. Angiotropic lymphoma with endocrine involvement.AIMS: Two cases of angiotropic high-grade B-cell lymphoma are described in which involvement of endocrine glands was a prominent feature. methods AND RESULTS: One patient had marked unilateral adrenomegally due to lymphoma within adrenal blood vessels. He is alive 5 years after presentation following combination chemotherapy. The diagnosis was made at autopsy in the second patient who presented with pyrexia of unknown origin and had hypothyroidism and a thyroid nodule. The thyroid gland and pituitary showed striking involvement by angiotropic lymphoma and the thyroid nodule was a cavernous haemangioma heavily colonized by lymphoma cells. CONCLUSIONS: Our cases illustrate endocrine organ manifestations of this rare disease. The condition may respond to combination chemotherapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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