Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience

Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor.     

Treatment for local control of retroperitoneal and pelvis sarcomas: A review of the literature

Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection with microscopically negative margins is challenging, and local recurrence rates can be high. Patients often succumb to uncontrolled local disease. Radiation therapy offers a potential means for sterilizing microscopic residual disease, although its use continues to be controversial. Chemotherapy alone or in combination with radiation continues to be investigated as an adjunct to surgery, along with immunotherapy and targeted therapies. In this review, we discuss the current management of retroperitoneal and pelvis sarcomas, focusing on studies of surgery and radiation therapy to maximize local control.     

Personalizing surgical margins in retroperitoneal sarcomas: an update

Introduction: Tumor biology, as well as completeness of surgical resection, are two important prognostic factors when treating retroperitoneal sarcoma (RPS). A frontline extended surgical approach is associated with improved local control and possibly improved survival. However, this approach has to be tailored to each histological subtype, as the patterns of growth and recurrence risks vary significantly among them.

Areas covered: We provide a review of the literature in RPS, describing the behavior of each of the five main histologic subtypes: well-differentiated liposarcoma (WDLPS), dedifferentiated liposarcoma (DDLPS), leiomyosarcoma (LMS), solitary fibrous tumor (SFT) and malignant peripheral nerve sheath tumor (MPNST). The prognostic factors relevant to oncologic outcomes of RPS, the role of margins and the importance of local control are discussed. Finally, a histologic specific surgical approach to RPS is provided in detail.

Expert opinion: While tumor-related factors are paramount, the only intervenable predictive factor is extent and quality of surgery. The extended surgical approach has been advocated for previously and again we describe it in more detail, tailored specifically to the tumor subtype. The aim of this approach is to maximize the possibility of achieving a complete resection through a standardized approach based on histologic behavior and site of origin.     

Pancreaticoduodenectomy in the surgical management of primary retroperitoneal sarcoma

Background

In retroperitoneal sarcoma (RPS), the optimal extent of resection must balance adequate disease control with potential for morbidity. We sought to study the frequency and outcomes after a Whipple procedure or pancreaticoduodenectomy (PD) in patients undergoing resection for primary RPS.

抗新生血管内皮细胞单克隆抗体BVE-1治疗实体瘤的实验研究

目的研究以肿瘤新生血管为靶,用抗血管内皮细胞单克隆抗体治疗实体肿瘤的可行性。方法裸小鼠移植人平滑肌肉瘤细胞,人肝癌细胞及人胰腺癌细胞,以抗血管内皮细胞的单克隆抗体ＢＶＥ１或１３１Ｉ标记抗体ＢＶＥ１腹腔给药,并与正常鼠ＩｇＧ及１３１ＩＩｇＧ治疗对比,观察肿瘤体积变化,死后及活杀鼠进行病理学检查,计算瘤内微血管密度。结果肝癌、胰腺癌、平滑肌肉瘤组经抗体ＢＶＥ１治疗后,抑瘤率分别为７０．５％、４８．７％和４９．８％,并抑制了平滑肌肉瘤转移和降低了死亡率。１３１Ｉ标记抗体ＢＶＥ１治疗组一次给药后抑瘤率达８６．６％～８２．２％,抑瘤生长率较单纯抗体组明显提高。病理检查证实,抗体ＢＶＥ１治疗组肿瘤区毛细血管管壁变性,管腔阻塞,周围肿瘤细胞大片坏死。瘤内微血管密度明显低于未治疗组及鼠ＩｇＧ组。结论抗增生血管内皮细胞抗体ＢＶＥ１具有杀伤及抑制肿瘤区血管内皮细胞生长作用,从而导致肿瘤区毛细血管阻塞,阻断血供,抑制了肿瘤生长和转移,降低了死亡率。以之为载体偶联核素可增强此作用。     

Laparoscopic Surgery for Anorectal Malignancies Other than Carcinoma

Objective:

Numerous concerns have been raised relative to the appropriateness of laparoscopic surgery for cure of rectal adenocarcinomas. However, because of their rarity, little information exists about the role of laparoscopy for other anorectal malignancies. We report the outcome of five patients who underwent laparoscopic surgery for other anorectal malignancies.

Methods:

All patients who underwent laparoscopic surgery for treatment of non-carcinomatous anorectal malignancy were assessed by means of endoscopic, radiological and histopathologic diagnostic tests.

Results:

Two patients with anorectal melanoma and one with anal leiomyosarcoma underwent laparoscopic abdominoperineal resection. A laparoscopic loop ileostomy was performed for an HIV-positive patient with rectal Kaposi''s sarcoma. Another patient with anorectal melanoma had intraoperative identification of distant liver metastasis and therefore underwent diagnostic laparoscopy instead of an intended abdominoperineal resection. There were no intraoperative laparoscopic complications. During the follow-up period three patients who underwent abdominoperineal resection were alive, one of whom had rectal melanoma and developed liver metastasis without local recurrence. The two patients with distant liver metastasis and rectal Kaposi''s sarcoma died 46 days and five months after surgery, respectively. There were no port-site or local recurrences.

Conclusion:

Laparoscopic abdominoperineal resection for non-carcinomatous anorectal malignancies is technically feasible and avoids many of the concerns associated with attempted curative laparoscopic resection of carcinoma.     

Leiomyosarcoma of the pancreas

We report a case of leiomyosarcoma of the pancreas. A spindle cell pattern at light microscopy, immunocytochemical reactivity with desmin, alpha-1-antitrypsin, vimentin and actin and ultrastructural features of smooth muscle differentiation help to establish the diagnosis.     

Pancreatic leiomyosarcoma

 A leiomyosarcoma originating from the pancreas of a 57-year-old man is presented. A 6×5×4 cm tumour was located in the head region, and the patient underwent surgical palliation. Immunohistochemical studies excluded an epithelial origin; a myogenic origin was suggested by strong vimentin and smooth muscle actin positivity. Flow cytometric analysis revealed an aneuploid pattern (DNA index: 1,561). The patient died with widespread metastases 7 month after the operation. Received: 11 November 1997 / Accepted: 2 February 1998     

Disseminated peritoneal leiomyomatosis with malignant change,in a male

Summary Disseminated peritoneal leiomyomatosis (DPL) is a rare disorder, characterized by the occurrence of multiple leiomyomas scattered throughout the peritoneal cavity. Until this report DPL had been observed only in women and there is only one previous case with malignant change. A case of DPL in a male, complicated by sarcomatous transformation is reported. DPL has a definite malignant potential; patients with the disease should be followed closely for signs of progression.