The primary retroperitoneal serous adenocarcinoma (PRSAC) is a rare malignant tumor of the retroperitoneum. It shares the same pathological and biological behavior with ovarian serous carcinoma. Most of the cases develop as peritoneal adenocarcinoma and rarely occur in the retroperitoneum. It is reported as serous surface papillary carcinoma of the peritoneum and extraovarian peritoneal serous papillary carcinoma. We present a case of PRSAC in a 60-year-old woman. Only 11 cases of PRSAC have been reported from 1983 to 2019. Histopathological features with immunohistochemical expressions are important to diagnose PRSAC. The outcome and survival mainly depend on the possibility of surgical resection. Molecular genetics of PRSAC should also be studied in relation with its ovarian counterpart.
A rare case of extrarenal Wilms' tumour in a Kadazan child is presented with a description of its clinical and pathological features. The possible origin of this tumour is discussed
Malignant fibrous histiocytoma is a mesenchymal tumour which can involve the genitourinary organs primarily or by secondary extension. Both conditions are rare. We report four cases of retroperitoneal malignant fibrous histiocytoma involving the kidney by local extension. Diagnosis was difficult because of diverse, non-specific clinical features and may only be reached at operation or post mortem. Prognosis is poor. Although en bloc tumour resection with nephrectomy was possible in two patients, they returned with recurrences.
A large retroperitoneal liposarcoma presented clinically as an ovarian tumour in an infertile woman. The importance of computed tomography in the differential diagnosis and the appropriate management of retroperitoneal liposarcoma is presented with a review of the relevant literature.
A case is reported of retroperitoneal malignant fibrous histiocytoma (MFH) invading into the colon, causing fresh bleeding per rectum. It illustrates the difficulty encountered in the pre-operative diagnosis of this condition, especially in a patient with an atypical presentation of profuse lower gastrointestinal bleeding.
We present a case of gross lower limb edema in a 21-year-old man with an intra-abdominal malignant fibrous histiocytoma. He had a 1-month history of lower limb edema secondary to inferior vena caval obstruction. His edema failed to respond to a combination of diuretics, oral frusemide 40 mg daily and oral spironolactone 100 mg daily. Subcutaneous drainage of both lower limbs with butterfly needles was performed with minimal improvement. However, he gained significant symptomatic relief with multiple subcutaneous punctures and stoma bag drainage. A total of 12.8 L of serous fluid was drained with this simple and effective method. This is the first report of the use of this method.
A ten-year review revealed a similarity in the incidence of teratoma in relation to other childhood tumors in Malaysian as compared with Caucasian children. The most common sites of origin were the sacropcoccygeal, gonadal, and retroperitoneal areas. The reason for the high incidence of retroperitoneal tumor in our series as compared with other countries is not clear. Late presentation and poor followup are associated with poor prognosis.
Extra-gonadal germ cell tumours (EGGCT) are rare. Therefore further investigations of the testis is aimed at sourcing a possible primary origin of gonadal tumour. Over the years, various case series on EGGCT have been reported questioning its true nature as in a majority of them, a primary source is found in the testis, thus representing a metastatic gonadal tumour. The testis pathology could be either a true germ cell foci, an intra-tubular epithelial neoplasia or an area of fibrosis, indicating a ‘burnt out tumour’. We report a 39-year-old male who underwent laparotomy and excision of a retroperitoneal tumour. Histopathological examination revealed retroperitoneal lymph node of mixed germ cell tumour origin. Clinical and ultrasound examination of bilateral testis was normal. The patient refused orchidectomy or a testicular biopsy. He underwent four cycles of bleomycin, cisplatin, and etoposide with no evidence of tumour recurrence on follow up and remains disease free after 12 months of diagnosis. A literature review of EGGCT, its relation and factors relating with future testicular tumour is presented.