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.