Centers of excellence or excellence networks: The surgical challenge and quality issues in rare cancers.

There are several suggestions that centralization of care improves outcome for rare cancers, particularly when optimal treatment requires complex surgery or high-technology radiotherapy equipment. Diagnosis and treatment in reference centers are expected to be more accurate because they benefit from large numbers of cases discussed in a multidisciplinary tumor board with a well-run pathway. However, centralization is sometimes moderately perceived by oncologists as a solution to be endorsed for rare cancer patients; disadvantages of centralization are the need for patients to move and the risk of a longer waiting list, with discomfort and possible negative effects on outcome. It is difficult to find single experts on rare cancers: all the more it will be difficult to find a multidisciplinary panel of experts, and the role of the surgeon is to be a functional part of it. On the other side, from a surgical point of view, the quality of the initial management of many rare cancers directly impacts the final outcome; surgery of rare cancers may not necessarily be more demanding than the average from a technical point of view, but the lack of cultural knowledge about the disease can well lead to inappropriateness even in the lack of major technical challenges. Care for rare cancer patients must be organized in pathways that cover the patient's journey from their point of view rather than that of the healthcare system, and pathways must follow the best evidence on diagnosis, treatment and follow-up.

Solitary pancreatic head metastasis from tibial adamantinoma: a rare indication to pancreaticoduodenectomy.

Pancreatic metastases are rare, <2% of all pancreatic neoplasia. This is the first case of pancreatic metastasis from adamantinoma, a rare, low grade and slow growing tumor which is frequently localized in long bones. We describe a case of a 45-year-old woman presenting with increased bilirubin level. Computed tomography and ecoendoscopic ultra sonography revealed a pancreatic head mass. Fine-needle aspiration biopsy was consistent with metastatic adamantinoma. The patient was submitted to a standard pancreaticoduodenectomy. As in the case presented, standard pancreatic resections are safe and feasible options to treat non-pancreatic primary tumor improving patient's survival and quality of life.

Intractable pain due to rectus abdominis intramuscular haemangioma.

Haemangiomas are tumours of vascular origin accounting for approximately 7 % of all benign tumours. Three types of haemangioma have been described according to the vessel type involved: capillary, cavernous and mixed. Intramuscular haemangiomas (IMHs) are infrequent, accounting for less than 1 % of all haemangiomas and are mostly located in the extremities and the trunk. Intramuscular haemangiomas of the rectus abdominis muscle are extremely rare, with only one previous case reported in the literature to the best of our knowledge. In this report, we present the case of a patient with intractable pain related to IMHs of the rectus abdominis and we analyse diagnostic assessment and surgical management of the condition.

Lymph-nodal ratio in gastric cancer staging system.

AIM: Many studies have indicated that lymph node metastases and the depth of invasion of the primary tumor are the most reliable prognostic factors for patients with radically resected gastric cancer. Recently the ratio between metastatic and examined lymph nodes (n ratio) has been proposed as a new prognostic indicator. The aim of this study was to evaluate the prognostic value of n ratio in patients with gastric cancer. METHODS: We retrospectively reviewed the data of 399 patients who had undergone radical resection for gastric carcinoma. RESULTS: N ratio was significantly greater in patients with large and undifferentiated tumors. Moreover, it was significantly related to both the number and location of lymph node metastases. Survival curves showed that n ratio was strictly related to patients' survival. Multivariate analysis confirmed that it was an important independent prognostic indicator. CONCLUSION: N ratio is useful to better evaluate the status of lymph node metastases in patients with gastric cancer submitted to radical surgery. Moreover it is a very important independent prognostic factor for gastric cancer.

Feasibility of the sentinel node biopsy in anal cancer.

AIM: Anal cancer is a rare neoplasm. According to a European Organization for Research and Treatment of Cancer multivariate analysis, synchronous inguinal lymph node metastasis occurs in 10-25% of patients and constitutes an independent prognostic factor for local failure and overall mortality. METHODS: Inguinal lymph node status was assessed using the sentinel node technique in 35 patients with anal cancer. RESULTS: Histology revealed 23 squamous carcinomas, 10 basaloid carcinomas, 1 squamous carcinoma with basaloid areas and 1 spinocellular epithelioma associated with areas of Bowen's disease. Disease stage was T1 in 5 patients, T2 in 18, T3 in 11 and T4 in 1 patient. Lympho-scintigraphy using a GE Millennium gamma camera was performed after peritumoral injection of 37 MBq of 99mTc colloid. Surgical sentinel node biopsy with a portable Scintiprobe MR 100 (Politech, Carsoli, Italy) had a detection rate of 97.1%. Inguinal metastases were detected in 7 (20%) patients, in 2 of which metastasis was bilateral. CONCLUSIONS: Given the correlation between prognosis and node involvement, sentinel node biopsy can be considered a simple method for adequate pretreatment staging of anal carcinoma. Use of the technique could avert the need for prophylactic inguinal radiotherapy in N0-N1 patients, thus reducing the morbidity associated with inguinal radiotherapy. Consistent follow-up is required to evaluate long-term results:

The role of sentinel lymph node biopsy in patients with differentiated thyroid carcinoma.

AIM: To evaluate the "state of art" of clinical role of sentinel lymph node (SLN) biopsy procedure in patients affected by differentiated thyroid carcinoma. METHODS: All papers cited on PubMed/MEDLINE until June 2005, published in English, and referred to the key words "sentinel lymph node biopsy" AND "thyroid carcinoma" OR "thyroid cancer" were reviewed for the purpose of the present study. RESULTS: The first method used for SLN biopsy in thyroid carcinoma patients was the vital blue dye technique. This technique had some disadvantages as: (a) risk of disruption of the lymphatic channels deriving from the thyroid cancer; (b) difficulty in disclosing SLN lying outside the central compartment; (c) parathyroid glands can take up blue dye and, thus, can be misinterpreted as lymph nodes. Some of the above cited disadvantages were overcome by using the lymphoscintigraphy and intraoperative gamma probe technique. A combination of the blue dye and gamma probe technique has also been proposed with synergic results. CONCLUSION: The reported advantages of the SLN biopsy in small differentiated thyroid carcinoma patients can be resumed as follows: (a) better selection of patients who would benefit from compartment oriented nodal dissection; (b) more accurate lymph node staging; (c) better selection of patients who can require (131)I treatment after surgery (SLN positive for metastasis); (d) better identification of SLN located out of the central compartment.

[Lymphoscintigraphic localization of sentinel lymph nodes in colorectal carcinoma in early stage: results of a single center study and proposal of a multicenter protocol]. / Localizzazione linfoscintigrafica del linfonodo sentinella nel carcinoma del colon-retto in stadio precoce risultati di uno studio monocentrico e proposta di un protocollo multicentrico.

In colorectal cancer the sentinel node dissection may help to identify any unusual mesenteric lymphatic drainage pattern from the primary tumor site (ex/skip metastases); assuming that accurate pathological staging is critical for therapeutic decisions we are conducing a study to evaluate the feasibility of the sentinel node technique in colorectal neoplasms and its overall accuracy in predicting regional lymph nodes metastases for appropriate staging. From February 2001 to September 2004 we included in this study 30 patients with rectal lesions or degenerate colonic polyps not radically excised by endoscopy. Lymphatic mapping was performed with low molecular weight albumin colloid labelled with 500Mci of 99mTc in a 2 ml volume and injected submucosally by an endoscopic route at the four cardinal points around the tumor, the afternoon before the surgical procedure, both in case of colonic or rectal lesions. Scintigraphic images were obtained with a gamma camera fitted with a general purpose collimator. The day of the intervention, a hand held gamma detecting probe (Scintiprobe m100, Pol-Hi-Tech, Italy) was employed to detect the "hot" nodes, in vivo and ex vivo. These lymph nodes were tagged with a stitch in vivo; the specimen was removed by a standard resection and SLN were dissected ex vivo and sent separately for pathological examination. In case of rectal lesions, the sentinel nodes were searched ex vivo into mesorectal fat in case. All lymph nodes, including blue or hot ones, were embedded separately for preparation of paraffin sections and haematoxylin and eosin staining. Sentinel lymph node were submitted to multi-seriate sections in order to look for micrometastases. Using the radioactive tracer, sentinel lymph nodes were successfully identified in 27 out of 30 patients. Concordance between SLNs and nodal status was observed in 23 out of 27 cases (85%); two patients (7.4%) were upstaged, as SLN was the only site of metastases. In another two cases we observed no concordance between negative sentinel node and non sentinel nodes (false negative rate, 7.4%). Starting from this experience we are proposing a multicentric trial concerning the value of sentinel node technique in rectal cancer and in early colorectal cancers detected by screening programs.

[Lymph node sentinel in gastric carcinoma: proposal of a multicenter GISCRIS (Gruppo Italiano per lo Studio della Chirurgia Radioguidata e dell'immunoscintigrafia) protocol]. / Linfonodo sentinella nel carcinoma gastrico: proposta di un protocollo multicentrico giscris (Gruppo Italiano per lo Studio della Chirurgia Radioguidata e dell'immunoscintigrafia).

Sentinel node biopsy has been widely adopted in the treatment of cutaneous melanoma and breast cancer. The ongoing controversy concerning the extension of lymphatic dissection in gastric cancer demonstrate that the optimal extent of lymphadenectomy has yet to be established, and underlines that the research in this area is needed to refine our knowledge and consequently our treatment of gastric tumors. In this paper the authors describe a multicentric protocol concerning the sentinel node research in early and advanced T1-T2 gastric cancer employing the blue dye method and lymphoscintigraphy by means of the endoscopic injection of Tc99m labeled nanocolloids. The aim of this protocol is to assess the clinical relevance of the sentinel node biopsy in selecting N+ patients in early gastric cancer, and the role of the same technique in detecting N2 positive patients in case of advanced gastric cancer. Assuming a confidence interval of +/- 5%, a sample of 100 recruited cases over three years is previewed.

Meta-analysis of non-sentinel node metastases associated with micrometastatic sentinel nodes in breast cancer.

BACKGROUND: The need for further axillary treatment in patients with breast cancer with low-volume sentinel node (SN) involvement (micrometastases or smaller) is controversial. METHODS: Twenty-five studies reporting on non-SN involvement associated with low-volume SN involvement were identified using Medline and a meta-analysis was performed. RESULTS: The weighted mean estimate for the incidence of non-SN metastases after low-volume SN involvement is around 20 per cent, whereas this incidence is around 9 per cent if the SN involvement is detected by immunohistochemistry (IHC) alone. Subset analyses suggest that studies with axillary dissection after any type of SN involvement result in somewhat higher estimates than studies allowing omission of axillary clearance, as do studies with more detailed histological evaluation of the SN compared with those with a less intensive histological protocol. Higher-quality papers yield lower pooled estimates than lower-quality papers. CONCLUSION: The risk of non-SN metastasis with a low-volume metastasis in the SN is around 10-15 per cent, depending on the method of detection of SN involvement. This should be taken into account when assessing the risk of omission of axillary dissection after a positive SN biopsy yielding micrometastatic or immunohistochemically positive SNs.

Radiolabeled localization of the sentinel lymph node: dosimetric evaluation in personnel involved in the procedure.

AIMS AND BACKGROUND: Peritumoral injection of 99mTc-labeled colloids for lymphoscintigraphy and radioguided surgery does not entail any relevant radiation burden to the patients. The real issue about radiation protection concerns the personnel involved in the procedure besides the nuclear medicine personnel. The aim of our study was to evaluate the cumulative doses to personnel involved during the injection of radiolabeled compounds, under ultrasound or stereotactic guidance and the radiation burden to the personnel involved in the surgical incision of the tumor 24 hours after the administration of 99mTc-labeled colloids. METHODS AND STUDY DESIGN: We performed environmental contamination tests (SMEAR TEST) and exposure evaluation in the operating room. RESULTS: In the operating room the removed activity in the analyzed samples was less than 0.5 Bq/g and exposure to the personnel was less than 6 micro Sv/h. The evaluations made during ultrasound guidance demonstrated an equivalent and effective dose less than 20 microSv. CONCLUSIONS: Our results show that during ultrasound or stereotactic administration of radiolabeled compounds the radiation burden to the personnel involved in the procedure is virtually negligible. The surgeons too are exposed to a negligible radiation dose.

The sentinel node in anal carcinoma.

AIMS AND BACKGROUND: Anal cancer is a rare condition. The inguinal lymph nodes are the most common site of metastasis in this neoplasm. The inguinal lymph node status is an important prognostic indicator and the presence of metastases is an independent prognostic factor for local failure and overall mortality. Depending on the primary tumor size and histological differentiation, metastasis to superficial inguinal lymph nodes occurs in 15-25% of cases. METHODS AND STUDY DESIGN: To evaluate the inguinal lymph node status we performed a search for the sentinel node in a female patient affected by squamous and carcinoma. RESULTS: Identification and examination of the sentinel node was positive and postoperative histology showed the presence of bilateral lymph node metastases. CONCLUSIONS: We suggest that examination of the sentinel node in anal cancer could be an efficient way to establish the inguinal lymph node status, which would help the clinician to plan and perform adequate treatment.

Sentinel lymph node and breast cancer staging: final results of the Turin Multicenter Study.

AIM OF THE STUDY: Validation of the sentinel node (SN) technique in breast cancer by means of lymphoscintigraphy. MATERIALS AND METHODS: From December 1996 to January 1999 102 T1-T2 breast carcinoma cases were recruited in Turin. 99mTc-human serum albumin colloids were injected subdermally the day before surgery (mean activity, 5.2 +/- 2.5 MBq). Scintigraphic imaging was performed after injection. After identification of the SN during surgery by a hand-held gamma probe, the SN was excised and sent for histologic examination. SN histology was compared with that of other axillary nodes. RESULTS: The SN detection rate was 86.3%; among 88 cases with an identified SN, 37 (42%) had axillary metastases; the SN was metastatic in 35 cases (sensitivity, 94.6%); in 51.3% of pN+ cases (19/37) the SN was the only metastatic site. In two of the 53 negative SNs, SN histology did not match with that of the remaining axilla (negative predictive value, 96.2%; staging accuracy, 97.7%). CONCLUSIONS: Our results agree with those reported in the literature; however, except in clinical trials and experienced structures axillary lymph node dissection should not be abandoned when mandatory for prognostic purposes, considering that at present SN biopsy alone is not completely accurate for axillary staging, especially in the absence of an adequate learning period.

[A case of presacral myelolipoma]. / Un caso di mielolipoma presacrale.

Myelolipoma is a rare benign pathology whose pathogenesis is still unclear. It is most frequently localised in the adrenal gland, followed by the presacral region, mediastinum, and perirenal and hepatic region. It varies in size from a few millimetres to several centimetres. The lesion is usually capsulated, detachable from the surrounding tissues and hypovascularised. In histological terms, it is composed of lipomatous and hematopoietic tissues. A 65-year-old female patient was referred to our unit after the finding of a nonbiopsied, pelvic mass during earlier surgery. The patient was in considerable pain owing to the extrinsic compression of the mass on abdominal and pelvic organs. Preoperative tests confirmed the presence of a voluminous presacral neoformation that appeared to have a possible medullary origin (chordoma or schwannoma). During surgery, the lesion was found to be capsulated and mainly adherent to the rectum. Histological analysis confirmed the myelolipomatous nature of the lesion. A review of 21 cases reported in the literature is presented.

Sentinel lymph node mapping and biopsy for breast cancer: a review of the literature relative to 4791 procedures.

The status of axillary nodes is the most important prognostic factor in breast cancer to select patient subgroups for adjuvant chemotherapy; the current standard of care for surgical management of invasive breast cancer is complete removal of the tumor by either mastectomy or lumpectomy followed by axillary lymph node dissection (ALND). The recent introduction of intraoperative lymphatic mapping and sentinel lymph node biopsy (SLND) represents a major new opportunity for appropriate and less invasive surgical management of many tumors. There is an almost uniformly enthusiasm concerning the potential of this technique in breast carcinoma management, shown by published data. A peculiar attention to the so-called "sentinel node debate" in breast cancer surgery is a constant in the last years issues of the major medical journals. Even patients have become more aware about medical enthusiasm and their request of concise information on the topic and the possibilities of this approach is an increasing reality in medical practice. The aim of this paper is to review recent literature to offer an overview about the main controversial methodological aspects and a wide analysis of reported results. The most significative international literature papers from Medline were retrieved from 1993 to September 1999, and 4782 procedures were analysed. This extensive review of the literature has confirmed accuracy, feasibility and reliability of the SN detecting technique in axillary mapping. Provided a good proficiency in SN localisation and pathological evaluation, human resources and efforts should be mainly focused on its clinical validation as an alternative to ALND instead of on further phase I-lI clinical studies.

Role of radioimmunolocalization in the staging of gastric carcinoma.

Intraoperative radioimmunolocalization is a potentially useful technique for staging gastric neoplasms without resorting to extensive surgical intervention. Before preoperative immunohistochemical typing for the presence or absence of tumor-associated glycoprotein (TAG) 72, we performed intraoperative radioimmunodetection on three patients presenting with gastric carcinoma using a whole monoclonal antibody (B72.3) marked with Indium-111 injected 1 week before operation. The results were calculated on the number of lymph node stations and yielded a high sensitivity due to a specificity of 72% and the absence of false negatives. Intraoperative radioimmunolocalization is a promising method for noninvasive staging of both early and advanced gastric carcinoma.

Sentinel lymph node biopsy and axillary staging of T1-T2 N0 breast cancer: a multicenter study.

From December 1996 to May 1998, 84 T1-T2 NO breast cancer patients were recruited for a multicenter study on the lymphoscintigraphic search of the axillary sentinel lymph node (SLN). The SLN was searched intraoperatively with a sodium iodide hand-held gamma-detecting probe (GDP) and excised before the standard axillary dissection was performed. Lymphoscintigraphy was unsuccessful in 8 of 84 cases (9.5%). In 73 of 76 patients with positive lymphoscintigraphy, SLN were found and excised (96%). The SLN proved to be predictive of axillary status in 71 of 73 cases (97.2%). Thirty of 41 patients had axillary metastases: in 16 cases, the SLN was the only site of the metastases (50%). In two cases, the SLN (reactive) did not match with the axillary status (2 of 63 reactive SLN, 4.6% of "skip" metastases). Age, tumor diameter, and histology seem to have little importance in affecting the predictivity of SLN biopsy. These results demonstrate the applicability of the lymphatic mapping techniques to a multicenter setting.