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We describe a patient with abdominal pain and ascites, mesenteric lymphadenitis and peritoneal panniculitis. Initially her ANA was negative. The abdominal pain recurred again three years later and in between the two episodes she had had skin rash, alopecia, arthralgia, and positive Coombs' test-haemolytic anaemia. Her ANA became positive a few years after the initial episode.
AIMS: Crystal-storing histiocytosis is a rare disorder described in patients with lymphoproliferative diseases, mainly in cases of multiple myeloma but also in lymphoplasmacytic lymphoma (immunocytoma). Most cases involve one single organ which, in the majority, is related directly to the presence of tumour. We describe a 44-year-old man with a clinical picture of multifocal fibrosclerosis (with mesenteric panniculitis, peritoneal, mediastinal and orbital fibrosis) in which the autopsy showed a systemic infiltrate of crystal-storing histiocytes and functional alteration of the organs involved, associated with IgG-kappa type immunocytoma. METHODS AND RESULTS: Histology showed a systemic infiltration, with a predilection for adipose tissue, by a diffuse cellular infiltrate composed of small lymphocytes, plasmacytoid lymphocytes and plasma cells, admixed with large number of crystal-storing histiocytes. Intracytoplasmic crystals were not identified either in the plasma cells or plasmacytoid lymphocytes. The neoplastic cells and the crystalline inclusions displayed reactivity with antibodies for IgG and the kappa light chain. A polymerase chain reaction study for the IgH gene showed a monoclonal rearrangement. Ultrastructural studies showed needle-shaped crystals surrounded by a single unit membrane. CONCLUSION: This case is, to the authors' knowledge, the first to be described in which crystal-storing histiocytosis is associated with a clinical picture of multifocal fibrosclerosis, which suggests that lymphoproliferative processes should be considered in the differential diagnosis of the various conditions associated with multifocal fibrosclerosis.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Mesenteric fibromatosis, sclerosing mesenteritis, inflammatory pseudotumor, and extrapleural solitary fibrous tumor constitute a loosely associated group of benign fibrous tumors and tumorlike lesions of the mesentery. These lesions are linked histologically by the presence of fibroblasts or fibrosis and anatomically by their location within the mesentery. Although rare, and distinctly different in pathogenesis and biologic behavior, these fibrous lesions have pathologic and radiologic features that overlap with one another and with more common neoplastic and nonneoplastic lesions of the mesentery. Mesenteric fibromatosis is a locally aggressive, benign proliferative process that may occur sporadically or in association with familial adenomatous polyposis. It most frequently manifests as a focal mesenteric mass and may simulate lymphoma, metastatic disease, or a soft-tissue sarcoma. Sclerosing mesenteritis is a rare idiopathic disorder that most commonly produces a stellate mass within the mesentery and should be differentiated from metastatic disease, specifically metastatic carcinoid, because it frequently responds to conservative or medical management. Inflammatory pseudotumor (inflammatory myofibroblastic tumor) is a benign, chronic inflammatory disorder of unknown cause that manifests as a solid mesenteric mass, indistinguishable from malignancy. Extrapleural solitary fibrous tumor is a tumor of submesothelial origin that is identical to the solitary fibrous tumor of the pleura. When located in the mesentery or peritoneal cavity, extrapleural solitary fibrous tumor has an imaging pattern that must be differentiated from metastatic disease, soft-tissue sarcomas, and other benign and malignant neoplasms of the mesentery and peritoneum. Knowledge of this group of benign fibrous tumors and tumorlike lesions of the mesentery is important in the preoperative evaluation of a mesenteric mass.
Mesenteric panniculitis is a rare condition of unknown etiology that causes inflammation of the adipose tissue in the mesentery. We describe a case in which mesenteric panniculitis was diagnosed on the basis of imaging and resolved spontaneously.
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PURPOSE: To evaluate the sonographic findings of mesenteric panniculitis (MP) and correlate them with CT findings. METHODS: We retrospectively evaluated the clinical, CT, and sonographic findings in 26 cases of MP in our hospital between January 1997 and July 2003. We also reviewed the sonographic features of MP previously described in the literature. RESULTS: The sonographic findings were well correlated to CT in 24 of 26 patients (92%). In these 24 cases, abdominal sonographic examination revealed a hyperechogenic, well-defined fatty mass (corresponding to the pseudocapsule CT sign) in the root of the mesentery, displacing the bowel loops. We found a clear interface between MP and normal intra abdominal fat. Examination with color Doppler sonography revealed the nondeviated mesenteric vessels within the mass. The persence of MP improved sonographic transmission in 9 obese patients and enabled the retroperitoneal vessels to be clearly visualized. Sonography was unable to demonstrate the preservation of the fat nearest the mesenteric vessels corresponding to the "fatty halo" sign on CT. CONCLUSIONS: Sonography is useful in the diagnostic workup for MP. The characteristic sonographic features of MP (well-defined mass, homogeneous hyperechogenicity of the mass, nondeviated vessels within the mass, and displaced bowel loops) correlate well with CT findings.
Sclerosing mesenteritis is a rare, usually benign disorder of the mesentery. Depending on the predominant tissue component (inflammation, fat, or fibrosis), it is known as mesenteric panniculitis or retractile mesenteritis. We present a rare case of retractile mesenteritis of the mesocolon as a cause of severe abdominal pain. US, CT, and MRI were the imaging modalities used. We emphasize the MR finding of a fibrous capsula in retractile mesenteritis, as this is to our knowledge the first study to describe this entity. This finding may be valuable for establishing a diagnosis of sclerosing mesenteritis, as well as for differentiating this disease from other mesenteric diseases.
Both inflammatory pseudotumors and sclerosing (retractile) mesenteritis are uncommon conditions of unknown origin. A number of authors have reported patients presenting with complaints that were attributed to one or the other of these entities. In our review of the literature, we were unable to find a single case report of mixed abdominal findings that could be explained by the presence of both conditions. We report a case of the patient presenting with abdominal mass that was identified as having features of both sclerosing mesenteritis and inflammatory pseudotumor on pathologic diagnosis.
A retrospective review of patients in our practice who underwent abdominal panniculectomy to facilitate gynecologic cancer surgery was performed. The objective of the study was to determine if panniculectomy was a safe and useful procedure in the morbidly obese gynecologic cancer patient. A total of 12 patients underwent the procedure between 1992 and 1996. Optimal pelvic oncologic surgery was accomplished in all 12 patients. All aspects of those procedures were performed by gynecologic oncologists. The Buchwalter retractor was used in all cases. The patients' weights ranged from 170 to 429 pounds, with a mean of 275 pounds. The mean body mass index was 48, with a range from 37 to 67. Four patients had a history of diabetes mellitus. Nine patients healed without wound complications. Three patients developed superficial subcutaneous wound infections/necrosis that were successfully managed with office debridement. Abdominal panniculectomy is a reasonably safe procedure that makes radical pelvic surgery possible regardless of the patient's weight. Prolonged wound bulb suction drainage may decrease the incidence of wound necrosis/infection in these high-risk patients.