Lipofibromatosis accompanied by several congenital anomalies, report of a rare case.

Lipofibromatosis is a rare tumor of infancy, which has been reported about 10 years ago, and since then very few reports have been published. This tumor has been reported in association with congenital anomalies such as macrosyndactyly, but there is no report of multiple congenital anomalies with lipofibromatosis in the English literature as far as our knowledge goes. Herein, we report a case of this tumor associated with syndactyly, bilateral complete cleft lip and palate, trigonocephaly, and atrial septal defect.

Unusual Thymic Hyperplasia Mimicking Lipomatous Tumor in an Eight-Year-Old Boy with Concomitant Pericardial Lipomatosis and Right Facial Hemihypertrophy

We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion.

Lipoma y Lipomatosis / Lipoma and Lipomatosis

Los lipomas son tumores benignos compuestos por células grasas. Ellos son los tumores mesenquimales más comunes. Se encuentran en el tejido subcutáneo y menos frecuente en órganos internos. Usualmente presentan poca dificultad de diagnóstico y morbilidad. Los lipomas típicamente se desarrollan como masas elásticas discretas en el tejido subcutáneo y tejidos blandos profundos en el adulto. Muchos signos cutáneos y algunos síndromes son anatómicamente muy complejos y pueden estar asociados con un lipoma subyacente. Recientemente un número de nuevas entidades y variantes han sido descriptas. Su reconocimiento es importante para evitar el diagnósitco fallido y terapias inapropiadas. Lipoma condroide, miolipoma y lipoma pleomórfico de células ahusadas puramente cutáneo, son neoplasias biológicamente benignas que pueden simular sarcomas morfológicamente. El advenimiento de las investigaciones moleculares y citogenéticas de las neoplasias lipomatosas tienden a contribuir más al entendimiento de la biología de aquéllas y guiar a la modificación de los esquemas de clasificación convencional.

Lipomas are benign tumors componed of mature fat cells. They are the most common benign mesenchymal tumor. Lipomas are found in the subcutaneous tissues and, less commonly, in internal organs. They usually present with little difficulty in diagnosis or morbidity. Lipomas typically develop as discrete rubbery masses in the subcutaneous tissues of the trunk and proximal extremity. Are the most common neoplasms of subcutaneous and deep soft tissues in adults. Most cutaneous signatures and someone syndromes are anatomically more complex and can associated with an underlying lipoma. In recent years a number of "new" entities and variants have been described. Their recognition is important to avoid diagnostic pitfalls and inappropriare therapy. Chondroid lipoma, myolipoma, and purely cutaneous spindle-cell/pleomorphic lipoma are biologically benign neoplasms which may mimic sarcomas morphologically. The advent of cytogenetic and molecular investigations of lipomatous neoplasms has contributed to a better understanding of the biology of these neoplasms and led to a modification of conventional classification schemes.

Adult paratesticular malignant fibrous histiocytoma treated with surgery, systemic chemotherapy and postoperative adjuvant radiotherapy.

Paratesticular malignant fibrous histiocytoma is an extremely rare malignancy of the scrotum. This malignancy has rarely been described in the literature and scant information exists on the optimal management of this cancer. We present here a case of a 57-year-old man with a diagnosis of high-grade malignant fibrous histiocytoma of the left intrascrotal region who underwent radical orchiectomy, systemic chemotherapy and postoperative radiotherapy.

Co-existent massive renal replacement lipomatosis and xanthogranulomatous pyelonephritis--a case report.

Renal replacement lipomatosis is a benign condition that may assume major clinical significance by producing pyelocalyceal deformities that may be mistaken for true renal masses. While the number of reported cases of renal replacement lipomatosis has been relatively small, this entity's prevalence is probably underestimated. We report a case of co-existent massive renal replacement lipomatosis and xanthogranulomatous pyelonephritis.

Association of ulcerative colitis with pulmonary sarcoidosis, subcutaneous lipomatosis and appendiceal adenocarcinoma.

We report a 52-year-old man with left-sided ulcerative colitis for 5 years and pulmonary sarcoidosis diagnosed 3 years back. He presented with subcutaneous lipomatosis and a right iliac fossa mass, which was diagnosed histologically as appendiceal adenocarcinoma. He was treated with right hemicolectomy, followed by chemotherapy.

Colo-colic intussusception due to intestinal lipomatosis.

Lipomas of the small intestine are usually found incidentally; symptoms occur in less than one-third of affected patients, especially when lipomas are more than 2 cm in size. We report a 32-year-old man in whom intestinal lipomatosis resulted in colo-colic intussusception. These symptoms disappeared following operative resection of about 60 cm of ileum and ascending colon.

Mediastinal widening in a patient of ulcerative colitis.

A case of ulcertaive colitis on long-term corticosteroid therapy presenting with mediastinal widening and diagnosed to have mediastinal lipomatosis an thoracic computed tomography is presented.

Segmental Jejunal Lipomatosis: A Rare Cause of Intestinal Obstruction

A rare case of a segmental small intestinal (jejunal) lipomatosis is described. A 33-year-old male was admitted with a clinical diagnosis of an acute intestinal obstruction. A plain erect abdominal x-ray showed multiple air-fluid levels. On an exploratory laparotomy, a jejunojejunal intussusception was found secondary to a segmental submucosal lipomatosis. This was treated by a segmental resection and anastomosis, which resulted in a complete cure. Here we present this case with a review of the relevant literature.

Compresión sacorradicular por lipomatosis lumbosacra / Symptomatic spinal epidural lipomatosis myelographic, CT and MR features

En un contexto de obesidad y/o corticoterapia prolongada, el incremento en el volumen del tejido adiposo epidural normal de la columna lumbosacra, puede ser causa de compresión sacorradicular simuladora de estrechez canalar o hernia discal. Entre 1986 y 1995 se estudiaron 6 pacientes con sacrorradiculografía, TC y RM. En todos los casos se observó hipertrofia de la grasa epidural responsable del cuadro compresivo

Shwachman-Diamond syndrome: the clinical imitador of cystic fibrosis

In this case we report the autopsy findings of a four-month-old boy with the diagnosis of Shwachman-Diamond syndrome. The clinical and pathologic findings are discussed with emphasis on the differential diagnosis of cystic fibrosis and a brief review of current literature

Extradural lipomatosis presenting with paraplegia.

An unusual case with spinal extradural lipomatosis in a non-obese and otherwise healthy man is reported. The patient presented with a history of weakness of legs which progressed to paraplegia over a 40 day period.

Co-existent renal replacement lipomatosis and pyelitis glandularis.

A 50 year old female was found to have a lump on the right side of abdomen with discharging wound on the same side and was ultrasonographically diagnosed as renal cell carcinoma. On histopathology, it showed replacement of the kidney by mature adipose tissue renal replacement lipomatosis, along with pyelitis glandularis which is a rare condition.

Von Hippel-Lindau disease without Lindau's tumour.

A case of Von Hippel-Lindau's disease lacking the classical Lindau's tumour is reported. The uncommon features of this case are highlighted and the relevant literature is briefly outlined.