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We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.

横紋筋肉腫は, 小児悪性腫瘍の中では代表的な疾患の一つであり, 組織学的には胎児型, 胞巣型, 多形型に分類されている.その中で胞巣型は10～25歳に多く, 頭頸部や四肢に好発する.今回われわれは15歳の女性の会陰部皮下に発生した胞巣型横紋筋肉腫の症例を経験したので, その細胞像を中心に報告する.腫瘍の穿刺吸引細胞診では, 腫瘍細胞は一部孤立散在性に, 一部ロゼット様に出現していた.細胞質は乏しく泡沫状で, N/C比は増大していた.核の多くは類円型で, 大きさはリンパ球の2～3倍であった.核縁の不整や切れ込みは散見されたが, 核縁の肥厚はみられなかった.核クロマチンは粗大顆粒状に増量しており, 核小体は小型だが複数のものも認められた.鼠径リンパ節の生検で得られた組織像では, 小型, 類円型の異型核を有する腫瘍細胞が胞巣状に配列し,「つるし柿」様の構造を呈しており, これらは細胞診におけるロゼット様の配列と対応していると考えられた.また, 免疫組織化学的にはデスミン, 横紋筋アクチンが陽性でケラチン, EMAが陰性であったため, 非上皮性の腫瘍であることが示唆された.また, フローサイトメトリーではDNA Index: 1.88の位置にDNA異数体が検出された.

巨大卵巣嚢腫のため,仰臥位での睡眠が困難な症例の周術期管理を経験した.術前にあらかじめ約35lの嚢腫内容液を排除したところ,仰臥位での睡眠が可能となった.手術を全身麻酔下に開始したが,嚢腫内容液の吸引により血圧は低下し,昇圧薬と急速輸液で対処した.同時に低酸素血症を呈したため,一時的に高濃度酸素吸入を行なった.術前排液量を含めた摘出臓器重量は53kgであった.術後は集中治療部にて管理したが,入室8時間後に血圧低下をきたした以外,呼吸・循環系は安定しており,術後2日目に集中治療部を退室し,後日軽快退院した.

明細胞が主体に構成される肺大細胞癌の1例を報告する.症例は86歳の男性.増量する喀疾を主訴に近医を受診し, 胸部X線写真上左肺上葉にcoin lesionを指摘された.気管支鏡下の気管支擦過細胞診を行いclassVと診断され, 左肺上葉切除術を施行された.気管支擦過細胞診所見は, 大小不同の著しい腫瘍細胞が, 比較的平面的に出現し, N/C比のばらつきも目立った.粗穎粒状のクロマチンを有し, 核小体の目立つ核と, ライト緑淡染性の淡明な細胞質を示した.切除肺には, 肉眼的に径3cmの黄白色境界明瞭な充実性腫瘍がみられた.病理組織所見では, 血管豊富な充実性腫瘍で, 明らかな扁平上皮癌および腺癌への分化は認めなかった.主体をなす細胞は, 細胞および核の大小不同が目立ち, グリコーゲンの豊富な明るい細胞質が特徴的であった.核の大小不同が非常に目立つ点と併せて, 細胞所見は組織所見をきわめてよく反映しているものと思われる.本邦における肺癌取扱い規約では, 明細胞癌は独立した組織型の存在が確認し難いとの理由で分類からは除外されている.WHO分類では肺明細胞癌は, 大細胞癌の一亜型として分類されており, その構成成分が淡明ないし泡沫状の胞体を有する腫瘍細胞であり, 腺細胞ないし扁平上皮への分化が認められていないものとして定義されており, そこに示されている基準を本例は満たしていると考える.腎細胞癌の肺転移を始め, 良性明細胞腫が鑑別診断として問題となったが, いずれも臨床検査上, また免疫組織化学的検索にて否定された.

Permanent tracheostomy and tracheoesophageal anastomosis were performed as a means of surgical intervention for the treatment of intractable aspiration pneumonia. Conventional methods of tracheoesophageal anastomosis have entailed various problems. The improved method devised by us uses the special histological features and enables safe and reliable anastomosis. By this method, the tracheal perichondrium is retained and the strength of the anastomosed portion of the trachea is maintained, the failure of sutures due to tension on the anastomotic site being prevented. Also, reanastomosis can be performed after the cure of primary disease.

Vascular endothelial growth factor A (VEGF-A) plays an essential role in tumor progression through stromal neovascularization in malignant solid tumors. Neuropilin (NRP) is considered to be the specific receptor for limited types of VEGF-A isoform, VEGF165. The clinicopathological implications of NRP are not well understood in colon cancer, while almost all colon cancers overexpressed VEGF-A. We examined the expression levels of NRP1 and NRP2 genes in 54 colon cancer cases and paired extraneoplastic tissue with quantitative real-time polymerase chain reaction. The gene expression levels of NRP1 in the tumor (0.431+/-0.583) were significantly decreased compared to those in the extraneoplastic tissue (0.754+/-0.799) (paired t-test, p=0.0208). On the other hand, the gene expression levels of NRP2 in the tumor (0.763+/-0.791) were not decreased compared to those in the extraneoplastic tissue (0.508+/-0.386) (paired t-test, p=0.0511). Twenty cases, with preserved expression of the NRP1 gene in the tumor, showed a better prognosis as compared to the 34 cases with decreased NRP1 expression (p=0.0258, log-rank test). No significant relationship was noted between NRP2 gene expression and prognosis. The results suggested that preserved NRP1 expression provides colon cancer patients with a better prognosis.

Abstract 2645 Background Programmed cell death-1 (PD-1) is involved in one of the inhibitory pathways of the B7-cluster of differentiation (CD) 28 family; this pathway is known to be involved in the attenuation of T cell responses and promotion of T cell tolerance. PD-1 is known to negatively regulate T cell receptor-mediated proliferation and cytokine production, lead to alternation in the tumor microenvironment. Carreas et al. (J Clin Oncol. 2009; 27: 1470–1476) examined 100 follicular lymphoma (FL) patients and reported better prognosis in the group that had high levels of PD-1-positive cells. In contrast, in the study performed by Richendollar et al. (Human Pathol. 2011; 42: 552–557), which involved 91 FL patients, high levels of PD-1-positive cells were found to have a poor prognostic impact. Although these studies have shown that high levels of PD-1-positive cells in FL patients influence their prognosis, both studies included patients treated without rituximab, and the prognostic impact of PD-1 positivity in the rituximab-era (R-era) has not yet been elucidated. Materials and methods We retrospectively analyzed data for 91 FL patients uniformly treated by standard rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) therapy in 5 institutions between 2001 and 2009. The median age of the entire cohort was 58 years (range, 34–85 years), and 46 (51%) of the patients were men. We also collected and examined biopsy specimens for diagnosis with respect to PD-1 positivity. The PD-1-positive cells were counted using computer analysis at the Cancer Institute, Japanese Foundation for Cancer Research. Results The FL grade on diagnosis was grade 1 for 34 (37%) patients, grade 2 for 41 (45%) patients, and grade 3 for 16 (18%) patients. The median positivity for PD-1 staining was 16.0% (range, 0.01–51.9%) and was significantly higher in the high beta-2 microglobulin (B2M; at least 3) group (P = 0.009); the men had a high tendency for PD-1 positivity (P = 0.08). After a median follow-up of 29.1 months, the 3-year progression-free survival (PFS) and overall survival (OS) were 61.1% and 88.6%, respectively. Stage 4 FL at diagnosis (P = 0.02) and bone marrow involvement (P = 0.05) resulted in worse PFS, and an Eastern Cooperative Oncology Group (ECOG) performance status of 2–4 (P = 0.04), high Follicular Lymphoma International Prognostic Index (FLIPI; P = 0.02), B symptoms (P = 0.04), and high B2M levels (P = 0.005) worsened OS. Multivariate analysis showed that age over 60 years (P = 0.04) and high B2M levels (P = 0.07) were prognostic factors for PFS. PD-1 positivity was not found to be a prognostic factor with respect to both PFS and OS. Because the addition of rituximab to therapy regimens has altered the clinical course and prognosis of FL, some new prognostic factors have been proposed, and the impact of known prognostic factors has been changing. Rituximab might also have changed the prognosis of FL patients with high levels of PD-1-positive cells. Conclusion High levels of PD-1-positive cells were not found to be a prognostic factor in this study, indicating that the prognostic impact of PD-1 positivity might be eliminated in the R-era. Disclosures: No relevant conflicts of interest to declare.