Parulekar SV

The retroperitoneal diseases are important because they can remain asymptomatic until late in the stage of the disease, can cause a lot of morbidity, are difficult to diagnose clinically, and surgical approach to their management can be quite challenging. Retroperitoneal fibrosis is one such disorder in which there is inflammation and fibrosis. It is generalized and spreading. It is of interest to the gynecologists when it involves the lower retroperitoneal space and obstructs the blood vessels and ureters running through it. It may be primary or secondary to tuberculosis, actinomycosis, histoplasmosis, exposure to drugs like ergotomines, methyldopa, hydralazine, bromocriptine, beta blockers, pergolide, and methysergide. It may also develop after trauma including surgery, and radiotherapy for cancer. These patients present with vague symptoms like poorly localized, dull pain in the back, flank, or lower abdomen, fever, edema feet, malaise, anorexia, nausea, vomiting, and loss of weight.  Diagnosis requires a high degree of suspicion and help of imaging techniques like ultrasonography, contrast enhanced computed tomography and magnetic resonance imaging. Definitive diagnosis is based on a biopsy which is not obtained easily. Treatment is with drugs, and is often not very satisfactory. Another retroperitoneal disorder of interest to a gynecologist is retroperitoneal and pelvic fibromatosis. It may be caused by trauma, exposure to drugs, and irradiation. It is often asymptomatic. It tends to be more localized than retroperitoneal fibrosis. It may present with gastrointestinal bleeding or obstruction, unilateral ureteric obstruction and hydronephrosis. Diagnosis requires imaging and biopsy. Its treatment is surgical excision. Drug therapy may also help. A third group of retroperitoneal disorders of interest to a gynecologist is tumors, which can be benign or malignant. They may be mesenchymal in origin (arising from fibrous tissue, skeletal muscle, fat, vessels and nerves), extragonadal germ cells, lymphoma, or adenocarcinoma. Common tumors include liposarcoma, leiomyosarcoma, fibrosarcoma, rhabdomyosarcoma, haemangiopericytoma, malignant peripheral nerve sheath tumor, and malignant fibrous histiocytoma. The sarcomas can become very large before they become symptomatic. The symptoms are vague and do not suggest the diagnosis. A retroperitoneal mass is not mobile on palpation, but that does not help much in clinical diagnosis because many large intraperitoneal masses are also fixed. Though imaging modalities help, a definitive diagnosis is possible only with a biopsy. Treatment is difficult and prognosis is often not good. We present two interesting cases in this issue – one of retroperitoneal inflammatory myofibroblastic tumor which presented as a vaginal mass, and another one of retroperitoneal and pelvic fibromatosis. I hope they prove of interest to our readers.