[Multiple lung metastases from cutaneous adenoid cystic carcinoma resected 21 years before].

A 79-year-old woman whose cutaneous tumor had been resected 21 years and 12 years (local recurrence)before pathologically confirmed as primary cutaneous adenoid cystic carcinoma (ACC), was referred to our hospital for the abnormal shadow on chest X-ray. Chest computed tomography (CT)revealed 3 nodules in the peripheral field of both lungs, which were diagnosed by echo-guided needle biopsy as metastasis from the cutaneous ACC, and were completely resected at 5 months intervals. Any recurrences have not been detected for 2 years after the lung resection. In primary cutaneous ACC, not only complete resection with adequate margin but long-term follow up is recommended.

A case of cytokeratin 20-positive large-cell neuroendocrine carcinoma of the breast.

We report a rare case of large-cell neuroendocrine carcinoma of the breast. A 63-year-old woman was admitted to our hospital with a firm mass in the right breast. Mammography revealed a high-density mass with specula, and ultrasonography showed a heterogeneous hypoechoic mass with irregular margin and posterior acoustical shadowing. She underwent breast-conserving surgery with axillary lymph node dissection. Histopathologically, the tumor consisted of large cells with polygonal and prominent nuclei and a small amount of eosinophilic cytoplasm, and these cells showed palisading arrangement with fibrous stroma. The tumor cells were positive for neuroendocrine markers such as NSE, synaptophysin, CD56 (NCAM), but not for chromogranin A. Interestingly, the tumor cells showed reactivity for cytokeratin 20 but not for cytokeratin 7. The immunostaining pattern is different from the usual adenocarcinoma of the breast. The patient received postoperative radiotherapy and was given adjuvant chemotherapy. She has remained disease-free for 44 months.

Intravesical mitomycin-C-induced interstitial pneumonia.

An 84-year-old man with no evidence of pre-existing interstitial pneumonia developed fatal interstitial pneumonia and respiratory failure that could only be explained as an adverse effect of intravesical mitomycin C chemotherapy. He had undergone transurethral resection of bladder cancer 3 times, followed by intravesical mitomycin C chemotherapy 1 month later. He had received intravesical mitomycin C chemotherapy every week for 2 months, and he had complained of dyspnea on exertion 5 days before the last intravesical mitomycin C chemotherapy session. Interstitial infiltration was detected in both the upper and lower lobes of the left lung on the day of the last instillation of mitomycin C. Despite discontinuation of mitomycin C and administration of methylprednisolone, his condition deteriorated and he died. Diagnostic evaluation of other causes of pneumonia, including infection and collagen-vascular diseases, was negative. The autopsy demonstrated diffuse alveolar damage in the lung; there were no remarkable findings in other organs. To our knowledge, this is the first pathologically confirmed case of fatal interstitial pneumonia due to intravesical mitomycin C chemotherapy.

Ovarian mucinous cystadenoma of borderline malignancy in a premenarchal girl.

Ovarian mucinous cystadenomas are benign epithelial neoplasms that occur most often in the third to sixth decade of life. Ovarian mucinous cystadenoma can be classified into 3 categories (benign, borderline malignancy, malignancy) based on histopathologic evaluation. Premenarchal cases of ovarian mucinous cystadenoma of borderline malignancy are exceedingly rare. To the best of our knowledge, there have been only 4 reported cases of borderline ovarian mucinous cystadenoma prior to 2009. Here we report a 13-year-old premenarchal girl with a giant mass occupying almost the whole of the abdomino-pelvic cavity.

Umbilical cyst containing ectopic gastric mucosa originating from an omphalomesenteric duct remnant.

An umbilical cyst originating from an omphalomesenteric duct remnant is extremely rare, and to the best of our knowledge, it has scarcely been reported in medical literature. We present ultrasonographic manifestations and computed tomographic findings of an umbilical cyst originating from an omphalomesenteric duct remnant in a 6-year-old girl. In cases of umbilical cyst, radiographic evaluation alone may not differentiate an omphalomesenteric duct remnant with heterotopic gastric mucosa from a urachal remnant, and surgical intervention is required.

Trisomy 10p and translocation of 10q to 4p associated with selective dysgenesis of IgA-producing cells in lymphoid tissue.

A combined chromosomal abberation trisomy of the short arm of chromosome 10 associated with translocation of 10q to chromosome 4p was found in a 14-month-old boy, who died after repeated bouts of pneumonia. The translocation involved the target region 4p16.3 of Wolf-Hirschhorn syndrome and/or Pitt-Rogers-Danks syndrome. The karyotype was 46,XY,der(4)t(4;10)(p16;q11.2),i(10)(p10),ish der(4)t(4;10)(p16.3;q11.2) (D4S96+,D4Z1+),i(10) (pter ++). In addition to growth retardation and external as well as internal dysmorphism, the patient had abnormalities of the immune system, such as thymic involution, generalized lymph node enlargement, unusual distribution of T cells in lymphoid follicles, and selective IgA deficiency. The IgA-producing cells were rarely found in lymph nodes but normally in intestinal mucosa. In contrast, in the lymph nodes, the paracortical T-lymphocytes were hyperplastic, but they rarely entered the primary follicles. It is assumed that the chromosomal abnormality may lead to the dysfunction of T lymphocytes and, further, to the dysgenesis of IgA-producing cells in lymph nodes but not in intestinal mucosa. This suggests that the thymus may differentially control the subsets of IgA-producing cells in lymph nodes and intestinal mucosa.

A case of nodular pseudoangiomatous stromal hyperplasia (PASH).

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a common microscopic lesion that is found at breast biopsy, and presents with proliferation of the stromal cells and slit-like pseudovascular spaces with endothelial-like spindle cells. In contrast, nodular PASH is relatively rare. We report here a case of nodular PASH with multiple palpable masses. A 49-year-old woman who had experienced gradual enlargement of her breasts for 13 years noticed an elastic but firm palpable mass in her breast. We were able to detect 7 masses in her right breast and 2 in the left. Ultrasonography and mammography demonstrated nonspecific findings, and FNA and CNB did not establish a diagnosis. An excisional biopsy was performed, and the pathological findings revealed nodular PASH. Eighteen months after the excisional biopsy, the size of the nodules and the whole breast had decreased remarkably. While the possibility of a change in the hormonal background or the influence of drugs was considered, we were not able to reach a single specific conclusion regarding the pathogenesis.

A case of ectopic hepatocellular carcinoma in the jejunum.

We describe the case of a 72 year-old man with a huge tumor in his lower abdomen and extremely high serum alpha-fetoprotein levels (99,100 ng/ml). The patient had no risk factors for hepatocellular carcinoma (HCC) or liver disease. Computed tomography, magnetic resonance imaging, and hepatic angiography detected no tumors in the liver before surgery. The arteries feeding the tumor arose from the superior mesenteric artery, but were not recognized on celiac angiography. Histologically, the tumor cells had features of HCC. Immunohistochemical staining revealed that the tumor cells were positive for AFP and the hepatocyte paraffin 1 monoclonal antibody. Furthermore, the tumor cells were strongly positive for cytokeratin 8 and cytokeratin 18, which are usually expressed on hepatocytes in HCC, and negative for both cytokeratin 7 and cytokeratin 20, which are not usually expressed in HCC. Hence, the tumor was diagnosed as an ectopic HCC that possibly developed from ectopic liver tissue in the jejunum. Approximately 2 months after the operation, transarterial chemoembolization was performed for liver metastasis from this tumor. One year after the transarterial chemoembolization procedure, the patient remains well with no evidence of a recurrent tumor or serum AFP elevation.

[A case of advanced gastric cancer with peritoneal dissemination responding remarkably to TS-1/CDDP combination chemotherapy].

A 57-year-old woman visited a physician with complaints of anorexia and pollakiuria. Because a pelvic tumor and ascites were detected, she was referred to our department. Douglas pouch puncture revealed adenocarcinoma cells. Further examination showed an advanced gastric cancer with peritoneal dissemination. The cancer was judged to be unresectable. Chemotherapy with a combination of TS-1 and CDDP was performed before the operation. After 2 courses of the chemotherapy, her complaints disappeared, although abdominal CT confirmed remaining peritoneal dissemination. After 7 courses of chemotherapy, abdominal CT showed that the peritoneal dissemination had disappeared. Total gastrectomy and lymph node dissection were performed. Histological findings of the stomach revealed complete disappearance of cancer cells in the stomach and the regional lymph nodes. We confirmed that the TS-1/CDDP therapy resulted in a complete response to advanced gastric cancer and peritoneal dissemination. We recommend that chemotherapy be continued until the peritoneal dissemination disappears.

[Tumor microembolism presenting as characteristic patterns of pulmonary perfusion on lung scanning: a case report].

A 55-year-old man presented with tumor microembolism manifesting as characteristic patterns of pulmonary perfusion on lung scanning. He had a 2-week history of dyspnea and general fatigue. Echocardiography demonstrated right ventricular enlargement. Computed tomography of the chest was normal. Lung perfusion imaging showed multiple subsegmental peripheral defects, which were characteristic of tumor embolism. Ultrasonography and computed tomography of the abdomen revealed multiple enlargement of the lymph nodes. Upper gastrointestinal panendoscopy showed gastric cancer. At 10 days after admission, he suffered cardiac arrest and died despite resuscitative efforts. Histological examination revealed pulmonary arterial obstruction with tumor cells, and poorly differentiated adenocarcinoma in the stomach and lymph nodes. This case emphasizes the need to include tumor microembolism in the differential diagnosis of dyspnea, even if there is no evidence of an underlying malignant tumor.

Microdistribution of alpha particles in pathological sections of tissues from thorotrast patients detected by imaging plate autoradiography.

Thorotrast is a colloidal suspension of radioactive (232)ThO(2) that naturally emits alpha particles (90%), beta particles and gamma rays (10%). Thorotrast was used as a radiographic contrast agent in the 1930s-1950s; it caused liver cancer several decades after injection because of its life-long deposition and exposure. Determination of the amount and the distribution of radioactive thorium are essential for assessment of radiation risks. We visualized alpha particles on ordinary archival tissue sections using an imaging plate and a BAS5000 image analyzer. Furthermore, we confirmed that the imaging system is sensitive enough to detect alpha particles and accurate in measuring the total amount of thorium deposited in the organ from a single tissue section. This method revealed that the amount of thorium deposited in tumor tissue is correlated to that in non-tumor tissue. Thorotrast deposition was not associated with DNA damage determined by histochemistry. In combination with histological findings, it is suggested that radioactive thorium always migrates within the deposited organs by macrophages, and that the organs are evenly exposed to alpha particles.