Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature.

BACKGROUND: Massive localised lymphoedema (MLL) is a rare, relatively recently described pseudosarcoma most often occurring in morbidly obese patients. AIM: To perform a retrospective review of all cases diagnosed as MLL. METHODS AND RESULTS: Clinical information was obtained. 22 morbidly obese adults (mean patient weight 186 kg) presented with unilateral, large soft tissue lesions of longstanding duration. Most lesions involved the thigh, but also occurred in the posterior calf and lower leg. Clinically, most lesions were regarded as representing benign processes, including pedunculated lipoma, lymphocoele or recurrent cellulites, although soft tissue sarcoma was also suspected in two cases. Grossly, all masses showed markedly thickened skin with a "cobblestone" appearance, and were ill-defined, unencapsulated, lobulate, and very large (mean size 31 cm, range 15-61.5 cm, mean weight 3386 g, range 1133-10,800 g). Histologically, all 22 cases showed striking dermal fibrosis, expansion of the fibrous septa between fat lobules with increased numbers of stromal fibroblasts, lymphatic proliferation and lymphangiectasia. Multinucleated fibroblastic cells, marked vascular proliferation, moderate stromal cellularity and fascicular growth raised concern among referring pathologists for atypical lipomatous tumour/well differentiated liposarcoma, angiosarcoma, and a fibroblastic neoplasm such as fibromatosis in 10, 2 and 1 case, respectively. CONCLUSION: The diagnosis of MLL continues to be challenging, in particular for pathologists. Awareness of this entity, clinical correlation and gross pathological correlation are essential in the separation of this distinctive pseudosarcoma from its various morphological mimics.

Fatal hemorrhage from a gastroaortic fistula secondary to gastric ulceration associated with Nissen fundoplication and nonsteroidal anti-inflammatory drug use.

Acute gastrointestinal hemorrhage from a gastroaortic fistula in the gastric fundoplication pouch is a rare complication of Nissen fundoplication. The present case reports a gastroaortic fistula secondary to gastric ulceration associated with prior Nissen fundoplication and nonsteroidal anti-inflammatory drug use. A 55-year-old man presented with massive hematemesis and died of exsanguination during emergency laparotomy. Recognition of factors that predispose a patient to gastric ulceration after fundoplication, including nonsteroidal anti-inflammatory drug use, is critical to arouse the high index of suspicion required to diagnose and manage this life-threatening complication.

Serial fMRI of adaptive changes in primary sensorimotor cortex following thumb reconstruction.

Serial fMRI was performed in a patient who underwent transfer of the left great toe to the hand following amputation of the right thumb. Motor activation within the primary sensorimotor cortex (SMC) of both hemispheres was quantified over 2 years, showing a transient increase in contralateral but not ipsilateral primary SMC. The temporal pattern of motor cortical activation observed in this patient may represent a "signature" of good functional recovery.

Thalamic relay site for cold perception in humans.

The neural pathways subserving the sensation of temperature are virtually unknown. However, recent findings in the monkey suggest that the sensation of cold may be mediated by an ascending pathway relaying in the posterior part of the thalamic ventromedial nucleus (VMpo). To test this hypothesis we examined the responses of neurons to thermal stimulation of the skin and determined the perceptual effects of microstimulation in the VMpo region in awake patients undergoing functional stereotactic surgery. In 16 patients, microstimulation in the VMpo region evoked cold sensations in a circumscribed body part. Furthermore, at some of these sites thalamic neurons were found that responded to innocuous cooling of the skin area corresponding to the stimulation-evoked cold sensations. These data provide the first direct demonstration of a pathway mediating cold sensation and its location in the human thalamus.