[Non-secreting paraganglioma retroperitoneal]. / Paragangliome non sécrétant rétropéritonéal: à propos d'une observation.

Non-functional retroperitoneal paragangliomas are rare tumors. They are often asymptomatic and can reach very large sizes. We report the case of a 49-year old woman with retroperitoneal tumor detected during CT scan examination performed to find the cause of non-specific abdominal pain. Malignant forms, more frequent than benign forms, show locoregional invasion and are characterized by delayed-onset metastases. The treatment of these tumors is based on the most complete degree of surgical resection, since prognosis depends on it. There is no consensus on the usefulness of complementary therapies which may nevertheless constitute a supportive treatment.

Response to neoadjuvant chemotherapy combined with regional hyperthermia predicts long-term survival for adult patients with retroperitoneal and visceral high-risk soft tissue sarcomas.

PURPOSE: To determine the efficacy of neoadjuvant chemotherapy combined with regional hyperthermia (RHT) for local tumor control and overall survival (OS) in adult patients with retroperitoneal or visceral (RP/V) high-risk soft tissue sarcomas (HR-STS). PATIENTS AND METHODS: From 1991 to 1997, 58 patients with HR-STS at RP/V sites were prospectively treated with four cycles of etoposide, ifosfamide, and doxorubicin combined with RHT followed by surgery, adjuvant chemotherapy, and radiation. RESULTS: Objective response rate assessable in 40 patients was 13% (five partial responses). Including minor responses (n = 8), the radiographic response rate was 33%. The pathologic response rate assessable in 26 patients after surgical resection was 42%. Median OS was 31 months. At a median observation time of 74 months, 5-year probability of local failure-free survival (LFFS), distant metastasis-free survival, event-free survival, and OS were 25%, 51%, 20%, and 32%, respectively. Averaged minimum temperatures (T(min)) and time-averaged temperatures achieved in 50% (T(50)) and 90% (T(90)) of all measured tumor sites differed significantly between responders and nonresponders (T(min), 39.3 degrees C v 38.0 degrees C; P =.002; T(50), 40.9 degrees C v 40.3 degrees C; P =.038; T(90), 40.1 degrees C v 39.3 degrees C; P =.017). At 5-year follow-up, probability of LFFS (59% v 0%; P <.001) and OS (60% v 10%; P <.001) was significantly in favor of patients responding to neoadjuvant thermochemotherapy. CONCLUSION: Response to neoadjuvant chemotherapy combined with RHT is predictive for an improved local tumor control resulting in a long-term survival benefit for patients with HR-STS at unfavorable RP/V sites; however, the impact of RHT has to be defined in a randomized phase III trial.

[Myxoid liposarcoma of the retroperitoneum. Presentation of a clinical case]. / Liposarcoma mixoide del retroperitoneo. Presentazione di un caso clinico.

Of all the soft tissue sarcomas, those localised in the retroperitoneum represent a particular challenge to the surgeon. Diagnosis is often late, when the tumour has already reached considerable proportions, creating particular problems for its removal. Liposarcomas are the most common histological form of retroperitoneal tumour. The authors report the case of a patient with retroperitoneal myxoid liposarcoma and use their analysis as the starting point for a review of the literature focused on the diagnosis and treatment of retroperitoneal tumours. They underline that modern imaging techniques (Eco, CT, NMR) are essential during the preoperative stage for a complete and detailed study of the neoplasm, as well as during follow-up. With regard to treatment, surgery is still the most effective approach in cases where this is feasible. The current trend towards aggressive surgery is backed by the improved results in terms of survival. An improved prognosis for patients with retroperitoneal tumours is linked to an early diagnosis and the development of more efficacious complementary therapies.

Retroperitoneal malignant fibrous histiocytoma causing variegated colonic lesions.

A 68-year-old man visited our hospital because of heartburn. A firm mass was palpated in the left upper abdominal quadrant. Ultrasonography and computed tomography revealed a large left sided retroperitoneal tumor. A barium enema examination showed shallow irregularly depressed or elevated lesions. Colonoscopy revealed an irregularly shaped ulcer and multiple submucosal masses suggesting invasion by an extrinsic malignant tumor. Although colonoscopic biopsy was negative, a resected tumor was histologically diagnosed as a malignant fibrous histiocytoma (MFH). When such varigated lesions are detected in the colon, MFH should be considered, and an attempt to sample the submucosal layer may be necessary.

[Retroperitoneal tumors: personal experience]. / I tumori retroperitoneali: esperienza personale.

The Authors experience in five retroperitoneal tumors with symptoms and histopathological characteristics not different from those described in the Literature, is here reported. Topographic findings, classification, and incidence of retroperitoneal tumors as referred by various Authors are discussed. Two aspects are particularly pointed out: the histological type and the surgical strategy for their removal. Concerning the first aspect, the Authors underline that benign lesion may have an optimal outcome and a long survival, unlike the malignant ones, which have always unfavourable prognosis, despite adjuvant and complementary therapy (radiotherapy, chemotherapy). As for the second aspect, surgery is the treatment of choice even when other organs different from those exclusively retroperitoneal, may be involved in the demolition.

[A case of retroperitoneal liposarcoma].

A case of retroperitoneal liposarcoma is reported. A 42-year-old woman was admitted to our hospital with the complaint of a palpable mass in her left abdomen. Intravenous pyelography, barium enema and an ultrasonogram revealed a huge left abdominal tumor. At operation, a huge yellow-brownish tumor found in the retroperitoneal space, was completely removed. The tumor was 32 x 24 x 12 cm and weighed 4,075 g. Histologically, it was a myxoid liposarcoma. Three courses of adjuvant chemotherapy (cis-platin) were administered. The postoperative course was uneventful and the patient has been free of disease for 16 months.

Barium peritonitis. Report of a case and review of the literature.

A case of generalized peritonitis, secondary to a perforation of the rectosigmoid colon during barium-enema roentgenography, is presented. The patient required immediate surgical intervention with the prime importance of the treatment being removal of as much of the contaminating materials as possible. This was done successfully with irrigation and wiping, using urokinase solution. Peritoneal lavage with urokinase solution was also carried out in the early postoperative period. Fluid replacement with careful monitoring of fluid and electrolyte balance is essential before, during, and after the surgical procedure. Adequate antibiotic therapy and careful respiratory and nutritional support are also important.

[A case of retroperitoneal liposarcoma].

A 77 year-old woman presented with the chief complaint of a mass in the right upper quadrant. The examination on admission including IVP, echography, Ga scintigraphy, barium enema, CT scan, and angiography revealed that the right kidney and ascending colon were displaced by the mass. Under the diagnosis of retroperitoneal tumor, operation was carried out. Most of the mass was resected with the right kidney. The pathological diagnosis of the resected tumor revealed well differentiated liposarcoma. Convalescence was uneventful and the patient has been well and free from disease for 14 months. Based on our experience and the review of the pertinent literature in English and Japanese, we emphasize the importance of definite surgery and close follow up, as liposarcoma is frequently recurrent.

[The enlarged retrorectal space--indicative of specific pathology or only unspecific symptom of disease in the true pelvis? (author's transl)]. / Der erweiterte Retrorectalraum--ein sicher pathologisches oder nur unspezifisches Zeichen einer Erkrankung im kleinen Becken?

The retrorectal space was measured on the lateral view of the rectum during double contrast barium enema examination in 300 patients in whom no abnormality of the rectum, sacrum or in the true pelvis was demonstrated clinically or radiographically. The measurement obtained is the distance between posterior wall of the rectum and the anterior surface of the sacrum between S2 and S5. The measurements at S5 are considered most accurate, since at this level the rectum will be positively in midline. The normal width at S5 is between 0.2 and 1.5 cm. In connection with other symptoms, a distance between 1.5 and 2 cm should be regarded as suspicious and above 1 cm as definitely abnormal, as was demonstrated in 95 cases with diffuse and localized enlargement of the retrorectal space. An abnormal measurement of greater than 2 cm however is an unspecific symptom and should not by itself be considered indicative of specific pathology, but rather should lend supporting evidence to other abnormal findings in the true pelvis.

[Retroperitoneal mesenchymomas. Remarks apropos of 19 cases]. / Les mésenchymomes rétro-péritonéaux. Réflexions à propos de 19 observations

The authors report 19 cases and review the literature. They analyse the pathological and clinical characteristics of retro-peritoneal mesenchymoma. The diagnosis of these tumours is facilitated by intravenous urography, barium enema, arteriography and bidimensional echography. Cavography is interesting to determine the venous risk. The unforeseeable course, which is always severe in these tumours, is characterised by the frequency of local relapse. The prognosis is very poor with only one 8 year survival in this series. Surgical removal is the only possible treatment, in spite of difficulty, especially during recurrences.