Tumori

Cystic peritoneal mesothelioma is a rare disease associated with a favorable short-term prognosis. Longer follow-up documenting a persistence of symptoms and a high rate of recurrence after debulking surgery along with an uncertain natural history prompt a re-evaluation of prior treatment recommendations. No prior long-term clinical study of these patients is available.

The experience with five cases of cystic peritoneal mesothelioma, four females and one male, are reviewed. All of these patients were treated with cytoreductive surgery with peritonectomy procedures and heated intraoperative intraperitoneal chemotherapy. CT, pathology and current status were investigated in order to learn more about the natural history of this disease.

All patients were symptomatic from abdominal distention and three of the four complained of severe pain. Female patients complained of long periods of recurrent abdominal and pelvic pain poorly managed by oral analgesics. In one patient prolonged conservative management over ten years resulted in transition to an invasive process with extensive lymph nodal metastases. Her prognosis for long-term survival is guarded because of mesothelioma extension into the chest. Disease control of both ascites and pain in the abdomen and pelvis was achieved in all five patients treated with cytoreductive surgery plus intraperitoneal chemotherapy.

Cystic peritoneal mesothelioma should no longer be referred to as “benign” cystic mesothelioma. An aggressive approach with complete disease eradication is the correct goal of treatment. From our experience, cytoreductive surgery to remove all visible tumor and intraperitoneal chemotherapy to control microscopic residual disease will help patients with peritoneal cystic mesothelioma to remain symptom- and disease-free over an extended time period with a single surgical intervention. Disease eradication may prevent the transition to an aggressive and fatal disease process.

Dermatofibrosarcoma protuberans is an uncommon, locally aggressive but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It may occur at almost any site but is most common in the trunk and extremities. Dermatofibrosarcoma protuberans of the breast has been rarely reported.

A 49-year-old woman without a significant medical history presented with a right breast mass that had been present for more than 10 years. Recently, progressive enlargement of the mass was noted. Physical examination showed a firm, well-defined, non-tender, mobile, erythematous 2 × 2 cm mass in the upper outer part of the right breast. Breast sonography showed a 2.3 × 1 cm slightly heteroechoic nodule with uneven anterior contours located in the dermal layer, highly suggestive of a dermal lesion. Wide excision of the mass was performed. Microscopically, the hypercellular tumor was composed of relatively monomorphic spindle cells forming a storiform pattern around the sweat glands of the deep dermis and the subcutaneous fat vacuoles. The tumor cells showed strong immunoreactivity for CD34. The final histological diagnosis was dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans of the breast, though uncommon, does exist. With the awareness of this entity can a prompt diagnosis be made and the disease be properly managed.

Somatostatin receptor scintigraphy (SRS) is the standard method for the detection of somatostatin receptors (SSTRs). It is commonly used in gastroenteropancreatic neuroendocrine tumor (GEP-NET) staging, and represents the criterion of choice for treatment with somatostatin (SST) analogs. Immunohistochemistry (IHC) was reported as a reliable method for the detection of SSTRs with theoretically superior sensitivity over SRS.

We retrospectively analyzed the sensitivity and specificity of IHC in the detection of SSTRs in a cohort of consecutive patients with GEP-NETs attending our Institute from 1997 to 2007. IHC analysis was restricted to SSTR2 and SSTR5, and the results were interpreted according to two different scoring systems. SRS was used as the gold standard.

Forty-four patients were enrolled; 24 (55%) had foregut carcinoids, 9 (20%) midgut carcinoids, 2 (5%) hindgut carcinoids, and 9 (20%) had GEP-NETs of unknown primary sites. A high concordance rate between IHC and SRS was shown, irrespective of the IHC scoring system applied (73% and 70%). The sensitivity of IHC was 89.3% and 78.6% and the specificity 43.8% and 50%, depending on the scoring system used.

Although SSTR2 was shown to be expressed by IHC in up to 50% of tumors not visualized by SRS, SRS still remains the method of choice in the diagnostic and therapeutic management of GEP-NETs. More pathological and clinical data are needed to properly understand the clinical relevance of immunohistochemical detection of SSTR expression in the absence of tumor uptake at SRS.

This report retrospectively analyzes 106 cases of endometrioid carcinoma of the ovary treated at the National Cancer Institute of Milan from 1974 through December 1993. In 12 of the 106 cases (11.3%) a synchronous carcinoma of the uterine body was observed.

Only patients who had previously untreated disease were included in the study. Patients with synchronous tumors were staged according to their ovarian cancer and treated according to the stage of that disease.

Thirty-nine patients (36.8%) had stage I, 17 (16.0%) stage II, 43 (40.6%) stage III, and 7 (6.6%) stage IV disease. Moderately plus poorly differentiated tumors were present in 76 of the 106 cases (71.7%). Considering the 67 patients with advanced disease, residual tumor was absent in 27 cases (40.3%), ≤ 2 cm in 17 (25.4%), and > 2 cm in 23 (34.3%) cases. Systematic pelvic and para-aortic lymphadenectomy was performed in 60 patients (56.6%); selective sampling was carried out in 23 cases (21.7%). After surgery, 77 patients underwent various chemotherapy regimens.

Using univariate analysis, FIGO stage, tumor grade, residual disease after surgery, lymph node status, and platinum in the chemotherapy regimen significantly influenced 5-year survival. However, when all these variables were included in a multivariate analysis only FIGO stage still had a significant impact on survival. Survival analysis also showed a trend towards longer survival in patients with synchronous tumors.

The pretreatment serum carcinoembryonic antigen (CEA) level is an independent prognostic factor in colorectal cancer. We aimed to investigate the significance of CEA as a prognostic or predictive factor in rectal cancer patients receiving preoperative chemoradiotherapy (CRT).

In total, 609 patients with locally advanced (cStage II-III) mid to distal rectal cancer who underwent preoperative CRT and radical surgery between 2001 and 2008 were analyzed retrospectively. Predictive factors for pathologic CRT response were determined using multivariate logistic regression. A prognostic factor analysis was performed using the log-rank test and Cox proportional hazards regression.

Elevated CEA levels (>5 ng/mL) were observed in 201 (33.0%) patients at diagnosis. Following preoperative CRT, downstaging (ypStage 0-I) occurred in 255 (41.9%) patients, of whom 88 had pathologic complete tumor regression. Pretreatment CEA was significantly associated with pathologic CRT response in terms of downstaging and tumor regression grade, and was the most relevant predictive factor. After a median follow-up period of 60 months, the 5-year disease-free and overall survival rates were 76.2% and 84.6%, respectively. Prognostic factors independently associated with recurrence or survival included ypStage, circumferential resection margin, and histologic grade.

In patients with rectal cancer who received preoperative CRT, the pretreatment CEA level was a significant and independent predictor of pathologic CRT response. However, it may not be able to predict long-term outcomes independently of ypStage.

Goserelin is a gonadotrophin-releasing hormone (GnRH) analogue that induces the suppression of gonadal steroidogenesis, and it could therefore be a medical alternative to irreversible surgical castration. We report the clinical and endocrine results from 40 goserelin-treated premenopausal patients with advanced breast cancer.

A depot formulation of the drug (3.6 mg s.c.) was administered fortnightly for the first 4 doses, and monthly thereafter. Gonadotrophins and estradiol (E2) levels were measured before and at specific times during the treatment.

Objective responses were observed in 17 of the 38 evaluable patients (45%), six of whom achieved complete remission. The best responses were observed on soft tissues (65%). Castration E2 levels were achieved in all of the patients.

Our results confirm that goserelin is as effective as surgical oophorectomy in premenopausal advanced breast cancer.

Sentinel lymph node biopsy has been accepted as a standard procedure for early stage breast cancer. In this retrospective analysis, the results obtained with different methodological approaches using radiocolloid with or without blue dye were examined.

A total of 158 sentinel lymph node biopsies were performed in 152 patients. Group A (85 patients) underwent lymphatic mapping using a combination of periareolar intradermal radiocolloid and subareolar blue dye injections. Group B (73 patients) underwent only periareolar intradermal radiocolloid injection. One large tin colloid and two small radiocolloids (nanocolloid of serum albumin -NC- and colloidal rhenium sulphide -CS-) were used.

Successful lymphatic mapping was attained in 157 of 158 procedures (99.4%). Radiocolloids localized sentinel lymph nodes in 99.4% and blue dye in 75.3% of the cases. The number of sentinel lymph nodes removed was greater in nanocolloid and colloidal rhenium sulphide groups ( P ≤0.05). Among 60 metastatic sentinel lymph nodes, frozen section analysis using hematoxylin and eosin staining failed to detect 1 macro- and 10 micrometastasis. Radiocolloid uptake was higher in sentinel lymph nodes accumulating blue dye (1643 ± 3216 counts/10 sec vs 526 ± 1284 counts/10 sec, P <0.001). Higher count rates were obtained by using larger sized colloids (median and interquartile range: tin colloid, 2050 and 4548; nanocolloid, 835 and 1799; colloidal rhenium sulphide, 996 and 2079; P = 0.01). Only 2 extra-axillary sentinel lymph nodes were visualized using periareolar intradermal injection modality.

Radiocolloids were more successful than blue dye in sentinel lymph node detection. More sentinel lymph nodes were harvested with small colloids, but different sized radiocolloids were similarly successful. Sentinel lymph nodes having higher radiocolloid uptake tended to accumulate blue dye more frequently. Sentinel lymph nodes manifested higher count rates when a larger colloid was used. Frozen section was very successful in detecting macrometastatic disease in sentinel lymph nodes, but the technique failed in most of the micrometastates.

Cystic mesothelioma is a rare disease that results in abdominal distention and poorly defined abdominal pain. Diagnosis has always been made by tissue biopsy rather than by radiologic studies.

Our experience with 7 patients with cystic mesothelioma includes 4 patients who had not had prior surgery before the performance of a high resolution CT scan. A special review of the radiology of these cases was performed in order to identify any pathognomonic signs of this disease.

In all patients who did not have a distortion of the radiologic images (as a result of surgical interventions) prior to the performance of a high resolution CT scan at our institution, a pathognomonic thin-wall cystic structure located within the gelatinous appearing mass was observed. These thin-walled cysts were of variable size and preferentially located within the greater omentum, pelvis and beneath the right hemidiaphragm.

Cystic mesothelioma can be diagnosed preoperatively by a high resolution abdominal and pelvic CT. The thin-walled cysts with great variation in size are located beneath the right hemidiaphragm, within the greater omentum and in the pelvis. No other disease with these radiologic findings has been identified.