黒田 誠

70代前半男性の肺動脈弁に発生したcardiac papillary fibroelastoma(CPF)の1例を報告する。健康診断にて心電図異常を指摘され受診したところ、肺動脈内に2.0×1.5cmの腫瘤を認めた。摘出標本のルーペ像では高度に乳頭状、絨毛状増殖した有茎性の腫瘤であった。組織学的に表面は一層の内皮細胞に覆われ、内皮下にはムコ多糖を含む疎な結合織があり、細胞密度の低い硝子化した芯を持つ三層構造を認めた。組織学的鑑別診断として粘液腫、ランブル疣贅が考慮された。CPFは今後、心エコー技術の発達や、スクリーニングの増加により、遭遇する機会が増える可能性がある疾患と思われる。CPFの臨床病理学的特徴について、文献的考察を加えて報告した。(著者抄録)

症例は60代男性。膀胱癌再発を疑って施行されたTUR-BT検体で、尿細管様構造を模倣する小型腺管が密に増殖しておりnephrogenic adenoma(NA)と診断した。術後定期的な経過観察がされていた5年後、再度同様の像を認めた。NAは生検診断時に尿路上皮癌や膀胱明細胞腺癌、浸潤性前立腺癌などとの鑑別が問題となることがあり病理診断医が知っておくべき良性病変の1つである。今回、膀胱尿路上皮癌の経過観察中に継続して認められた膀胱の多発NAの1例を経験したので報告する。(著者抄録)

We report what to our knowledge is the first documented case of "peripheral T-cell lymphoma-not otherwise specified" (PTCL-NOS) associated with a rectal adenoma. Colorectal lymphomas are relatively uncommon, but colorectal T-cell lymphomas are extremely rare. The patient was a 92-year-old woman, referred to our hospital for investigation of anal bleeding. Rectal examination revealed a 3-cm pedunculated polypoid mass 5 cm above the anal verge. We performed a transanal local excision, and histopathological examination revealed a tubulovillous adenoma, with the diffuse proliferation of large lymphoid cells mainly discernible in the mucus membrane lamina propria, with infiltration to the submucosal layer. Immunohistochemical examination of the lymphoid cells revealed positive staining for CD3, weak staining for CD4 and CD8, and negative staining for CD5, CD10, CD20, CD56, CD79a, TIA-1, and Granzyme B. The tumor was diagnosed as PTCL-NOS associated with rectal tubulovillous adenoma. The patient died 28 days after surgery, most probably of progression of the PTCL-NOS.

ベセスダシステム2001における異型腺細胞(Atypical glandular cell、以下AGC)は低頻度かつ細胞形態学的検討が十分でなく、その臨床的取り扱いについては苦慮することが多い。今回、2009年4月から2011年10月までに当院にて子宮頸部細胞診を行った4256例中AGCと診断された23例(0.54%)について、その組織学的所見と取り扱いについて後方視的に検討した。AGC症例23例の年齢中央値は45歳(27〜76歳)で、AGC-not otherwise specified(NOS)が21例、AGC-favor neoplastic(FN)は2例であった。16例にコルポスコピーが施行され、NCF 6例、ACF 10例であった。組織学的診断の内訳は、高度病変(CIN2以上の異形成、上皮内癌および浸潤癌)8例、CIN1 3例、良性または病変を認めなかった症例9例、その他3例(頸部扁平上皮癌同時化学放射線療法(CCRT)後1例、頸部扁平上皮癌CCRT後再発1例、および進行乳癌1例)であった。高度病変の詳細は頸部腺癌4例、AIS 1例、頸部腺扁平上皮癌1例、子宮体部類内膜腺癌2例であった。AGCには多岐にわたる高度病変が含まれている可能性が高く、AGC診断後に漫然と子宮頸部細胞診とHPVテストを繰り返すことは病変検出の感度が低く推奨されない。取り扱いはASCCPのガイドラインに準拠する。AGC-NOS、AGC-FNともにコルポスコピー、頸管内生検、HPVテスト、35歳以上では内膜生検が推奨されている。AGC-FNの症例で、精査により明らかな病変が明確ではない場合、積極的に診断的切除術を考慮すべきである。また、AGCと診断された症例に対し、p16やCarbonic Anhydrase IX(CA-IX)などの免疫細胞化学を併用することにより、診断精度の上昇が期待される。(著者抄録)

39歳、女性。初診の4ヵ月前に統合失調症で入院中の精神病院において、左大腿部内側の小皮下腫瘤を指摘された。経過観察していたところ4ヵ月間で急激に増大し当科転院となった。左股部から大腿部内側にかけて約20cm大の巨大な皮下腫瘤を認め、中央部は自壊し、著明な悪臭を伴っていた。腫瘍から多量の出血を繰り返しており、歩行不能な状態であった。画像検査では深部は薄筋まで浸潤していたため、筋肉を含めて全摘し遊離植皮術を施行した。腫瘍の全摘出により全身状態は改善し歩行も可能となるなどQOLの改善が得られた。現在術後8ヵ月で局所再発は認められていないが、肺転移を認めている。全摘標本の病理組織所見では小円形の腫瘍細胞が不均一に増殖しており、腫瘍細胞はMIC2およびPAS陽性であり、骨外性Ewing肉腫が最も考えられた。(著者抄録)

We report three cases in which umbilical endometriosis developed after a laparoscopic subtotal hysterectomy (LSH) for uterine myoma without endometriotic lesions.<BR>Case 1: A 36-year-old, gravida 2, para 2 underwent an LSH for uterine myoma. Removal of the uterus was performed using the peeling technique with an umbilical trocar. Twelve months postoperatively, induration appeared at the umbilical region (approximately 20 mm) and the right trocar region (approximately 5 mm). Therefore, a lumpectomy was performed 18 months after the initial surgery. The pathologic diagnosis was heterotopic endometriosis. Because the umbilical tumor relapsed 27 months following surgery, Dienogest was administered; eight months later, the tumor had resolved. The umbilical tumor reappeared two months after terminating the orally administered treatment; therefore, resection of the umbilical tumor was performed 55 months after the initial surgery.<BR>Case 2: A 44-year-old gravida 3, para 2 underwent an LSH for uterine myoma. The tumor was morcellated using a morcellator, then extracted using an umbilical trocar. Approximately 19 months postoperatively, an approximately 18 mm area of induration appeared near the umbilical region; therefore, a resection was performed. The pathological diagnosis was heterotopic endometriosis.<BR>Case 3: A 45-year-old gravida 2, para 2 underwent an LSH and left salpingectomy for a uterine myoma and left paraovarian cyst. The hysterectomy involved morcellation and extraction through the umbilical region. An approximately 46 mm area of induration with hemorrhage appeared in the umbilical region 70 months postoperatively. Due to the suspicion of an umbilical tumor, a needle biopsy was performed at the site; the pathologic diagnosis was heterotopic endometriosis. She is currently receiving Dienogest treatment as an outpatient, and is being followed-up with the possibility of surgical extraction in mind.<BR>Conclusion: Although endometriosis is a common gynecological disease, umbilical endometriosis is rare. Therefore, the disease that developed in these three cases was likely due to growth of endometrial tissue in the umbilical region at the time of tissue removal.

症例は56歳女性。主訴は歩行時呼吸困難、下痢。既往歴に気管支喘息、肺炎、入院時現症で両下肢に紫斑と網状皮斑を認めた。上部消化管内視鏡検査で十二指腸球部にびらん、発赤、浮腫を認めた。下部消化管内視鏡検査で全結腸にアフタ様潰瘍が多発、S状結腸に不整形・地図状潰瘍も認められた。いずれの病変粘膜の生検組織でも粘膜下層に好酸球浸潤を認め、下肢の紫斑の生検組織では真皮内の小動脈壁にフィブリノイド壊死と内弾性板の破壊が認められた。これらよりアレルギー性肉芽腫性血管炎と診断しステロイドと免疫抑制薬による治療を開始。その後呼吸困難、下痢は改善し全結腸に認めたアフタ様潰瘍も消失、S状結腸の潰瘍も瘢痕化していた。(著者抄録)

Alveolar soft part sarcoma (ASPS) that originates from the uterine cervix is extremely rare, with only thirteen cases reported. The participation of the ASPL-TFE3 chimeric gene, translocation (X; 17) (p11; q25), has been demonstrated in ASPS. Here, we report a case of cervical ASPS, along with a review of the literature. The patient, a 56-year-old woman, was referred for a 70 x 80 mm cervical tumor. She underwent a hysterectomy and bilateral salpingo-oophorectomy, and remained disease free for 66 months without adjuvant therapy. Pathological examination revealed features consistent with ASPS. In addition, the present case demonstrated strong positive nuclear staining for TFE3, and ASPL-TFE3 fusion gene type 1 was detected by RT-PCR. In a review of fourteen cases of this tumor (including the present case), the immunohistochemical expression patterns of myogenic or neuroendocrine markers were somewhat varied among cases. In all cases except for the present case, the patients were under 40 years of age, and the tumor sizes were under 5 cm. The prognosis of ASPS in the cervix was considerably better than that of ASPS in soft tissues. Complete resection with adequate margins is thought to be important, although the appropriate surgical method, including lymph node dissection, is uncertain. The role of chemotherapy or radiotherapy as adjuvant therapy has not been defined. Cervical ASPS is extremely rare, making case series the most viable option for understanding their natural history and for developing a treatment strategy, including an optimal surgical procedure and adjuvant therapy.

Transformed sarcomas rarely arise from bone infarct lesions, although the majority of bone sarcomas are primary in origin. However, the pathogenesis of the condition is unknown. In this report, we describe a malignant fibrous histiocytoma with a p53 gene mutation. A 59-year-old woman complained of having pain in her left knee for three months. Plain radiographs of the distal metaphysis of her left femur revealed an ill-defined lytic lesion, which was consistent with a malignant tumor in the infarct lesion. An open biopsy specimen did not show any evidence of malignancy. Immunohistochemical examination of the biopsy specimen failed to show p53 protein-positive cells. However, a mutation in the p53 gene was detected when polymerase chain reaction/single-strand conformation polymorphism (PCR-SSCP) analysis was performed. A functionally relevant p53 missense mutation in codon 273 of exon 8 [CGT (Arg) -&gt; CAT (His)] was confirmed by direct sequencing. We concluded that this lesion was a malignant bone tumor arising from the bone infarct lesion, and we thus performed a wide resection. The histopathological diagnosis of the resected specimen was that it was a malignant fibrous histiocytoma associated with bone infarction. Immunohistochemistry revealed that the tumor cells were positive for the p53 protein. To our knowledge, our patient is the first patient having a bone infarct-associated sarcoma with a p53 gene mutation. Identification of the p53 mutation helps in diagnosing the malignant transformation of the bone infarct lesion. One pathogenesis of this condition may be a mutation in the p53 gene.

Primary uterine cervical neuroendocrine tumors are rare, but affect relatively young women and the prognosis is poor despite multidisciplinary treatment. The incidence of meningeal carcinomatosis arising from malignant tumors of the uterine cervix is extremely low, only two patients with meningeal carcinomatosis arising from a uterine cervical neuroendocrine tumor have been reported in the English literature. Moreover, there have been no reports in which this was confirmed at autopsy. We encountered a pregnant woman aged 33 years who was diagnosed as having atypical carcinoid of the uterine cervix after radical surgery. Despite multidrug chemotherapy (paclitaxel + etoposide + cisplatin and irinotecan + carboplatin), the patient developed multiple organ metastases. Although there was no metastasis to the brain parenchyma or the spinal cord parenchyma, the patient also developed meningeal carcinomatosis. Whole-brain radiation therapy was performed, but was ineffective. The patient died at 19 months after her initial operation and 10 days after diagnosis of meningeal carcinomatosis. The presence of meningeal carcinomatosis was confirmed at autopsy.

副甲状腺ホルモン関連蛋白（PTH-rP）は悪性腫瘍に伴う高Ca血症の原因として知られている．症例は78歳男性．1カ月前からの食欲低下，腰痛で発症した．高カルシウム血症を呈し，画像所見から肝臓，脾臓，腰椎・肋骨への多発転移巣が認められた．いずれも病理所見上アポクリン腺癌の転移であり，陰嚢部原発巣を含め免疫組織染色でPTH-rP産生が確認された．アポクリン腺癌によるPTH-rP産生の報告はなく，貴重な症例と考えられた為，報告する．<br>

Interaction of CA19-9 with E-selectin is involved in initiation of hematogenous metastases. We investigated whether serum concentrations of E-selectin and CA19-9 are good predictors of hematogenous metastases and prognosis in colorectal cancer. Pre-operative serum samples were obtained from 152 patients with colorectal cancer, and from 28 healthy volunteers. Correlation between serum E-selectin and CA19-9 was studied in terms of clinically detected hematogenous metastases and prognosis of patients. Low serum concentration of E-selectin was defined as &lt; 50 U/ml in healthy volunteers, and on that basis, 20.4% of all patients belonged to the high E-selectin group. Several distinctive characteristics were observed in the clinical course of patients with high serum concentrations of both CA19-9 and E-selectin. The rate of Dukes&apos; D cancer was significantly higher, and curative surgery was performed less frequently in patients with high serum concentrations of both CA19-9 and E-selectin (60 and 40%) than in others. However, there was no significant difference in the frequency of recurrence after curative surgery between patients with high serum concentrations of both CA19-9 and E-selectin (25.0%) and others. Overall the 5-year survival rate was significantly lower in patients with high serum concentrations of both CA19-9 and E-selectin (34.3%) than in other patients. Even if the serum concentration of CA19-9 was high, prognosis was not poor in patients with low serum concentration of E-selectin. These results suggested that it was useful to measure both CA19-9 and E-selectin as markers of hematogenous metastases and as predictors of prognosis in colorectal cancer.

Background: Therapeutic strategies for flat elevated (0-IIa) lesions in the stomach diagnosed as adenoma by biopsy are currently not established, because some difficulties have previously been reported in the evaluation of vascular patterns alone for the differential diagnosis between adenoma and carcinoma. Objective: We attempted to evaluate the 0-IIa lesions diagnosed as adenoma by using magnifying endoscopy with narrow-band imaging (MENBI) to distinguish them as either adenoma or carcinoma. Setting: Department of Gastroenterology, Fujita Health University. Patients: Fourteen adenomatous lesions (6 adenomas and 8 carcinomas confirmed postoperatively) diagnosed with preoperative biopsies from patients who had undergone endoscopic submucosal dissection were evaluated. Interventions: We selected 5 sites per lesion for MENBI. Selected sites were divided into superficial structures (SSs) and irregular microvascular patterns (IMVPs). Main Outcome Measurements: The rate of SSs and IMVPs in adenoma and carcinoma. Results: Significant SSs were tubular in the adenoma and unclear in the carcinoma. Regarding IMVP subcategories, (1) slight intrastructual irregular microvascular patterns (ISIMVPs) accounted for 97%, (2) severe ISIMVPs accounted for 0%, (3) Fine networks accounted for 3%, and (4) corkscrews accounted for 0% of cases in the adenomas. The corresponding proportions in the carcinomas were (1) 40%, (2) 15%, (3) 45%, and (4) 0%. Severe ISIMVPs and fine networks were significant Findings for carcinomas. Limitations: The number of cases was limited. Conclusions: Our combined evaluation method using MENBI offers the ability to establish proper therapeutic strategies for lesions that are difficult to identify as adenoma or carcinoma.

今回我々は、胆管癌と鑑別が困難であった原発性硬化性胆管炎の1例を経験したので報告する。症例は73歳の女性、高血圧症で通院中の医院で肝機能障害を指摘され、腹部超音波検査で肝内胆管拡張を認めたため当院に紹介となった。入院時には黄疸は認めなかったが、入院中にT-bil 8.8mg/dlと黄疸が出現したため内視鏡的逆行性胆道造影(endoscopic retrograde cholangiography:ERC)を施行した。ERCでは左右肝管から総肝管にかけて狭窄像を認めた。胆汁細胞診、ブラッシング細胞診では悪性所見はなかったが、経乳頭的胆管生検で高分化型腺癌を否定できない所見を認めたため経皮経肝門脈塞栓術を行った後、肝左3区域切除術+D2リンパ節郭清を施行した。術後病理診断は胆管周囲に炎症細胞の浸潤と線維化による壁肥厚を認め原発性硬化性胆管炎と診断された。(著者抄録)

Tall cell variant (TCV) of papillary thyroid carcinoma is a rare tumor, which is usually associated with poor outcome, and pathologists often face the dilemma of proper diagnosis of TCV, not only by cytology but also histology. To allow surgeons to determine aggressiveness of the tumor before operation, it is important for pathologists to detect tall cell features correctly by, fine-needle aspiration cytology (FNAC). However, the current criteria and definition of TCV are still controversial and confounded by another problem, the differential diagnosis from poorly differentiated thyroid carcinoma (PDC). In this report, we describe two cases of TCV and present characteristic FNAC findings. The tumor cells had a peculiar shape, which included tall, columnar, and oxyphilic cytoplasm with "eccentric," basally located nuclei. We propose new, terms for these cells, such as "tail-like cells" or "tadpole cells." In the surgically-resected specimens, both cases exhibited remarkable extrathyroidal invasion accompanying prominent vascular invasions. They showed high Ki-67 (MIB-1) labeling index by immunohistochemistry, which indicated a higher proliferation activity of TCV than conventional form of papillary thyroid carcinoma. Furthermore, we discuss in this report the problematic issue of differential diagnosis of TCV from PDC and oxyphilic papillary thyroid carcinoma. Diagn. Cytopathol. 2009;37:732-737. (C) 2009 Wiley-Liss, Inc.