[A Case of Metachronous Quintuple Cancer Involving the Stomach, Rectum, Colon, Liver and Prostate].

Advances and improvements in cancer diagnosis and treatment have made it possible to find multiple primary cancers. We report here a rare case of metachronous quintuple cancer involving the stomach, rectum, colon, liver and prostate. An 80s‒ year‒old male was referred to our hospital with abnormality on upper GI series. He had undergone a distal gastrectomy in May 2005. Postoperative diagnosis was advanced gastric cancer (pT2N1M0, pStage ⅡA). In August 2006, anterior resection was performed with a diagnosis of advanced rectal cancer(pT3N0M0, pStage Ⅱa). For ascending colon polyps, endoscopic submucosal dissection was performed with a diagnosis of adenocarcinoma in adenoma(pTisN0M0, pStage 0)in September 2007. In June 2016, laparoscopic ileocecal resection was performed with a diagnosis of advanced cecum cancer(pT3N0M0, pStage Ⅱa). Follow up CT images showed a liver tumor in S4. Partial liver resection was performed in October 2010. Postoperative pathological diagnosis was hepatocellular carcinoma(pT2N0M0, pStage Ⅱ). Prostate cancer(cT2aN0M0)was treated by androgen deprivation therapy from February 2018. Although he had high‒frequency microsatellite instability, germline mutations in hMLH1 and hMSH2 genes were not detected. Histopathological examination showed that each tumor was an independent tumor and had not metastasized from any others. The patient had a good clinical course after these treatment until now.

[A Case of Solitary Fibrous Tumor of the Liver].

Most solitary fibrous tumors(SFT)occur in the thoracic cavity and reports on liver SFT are very rare. We encountered a case of SFT of the liver, and thus, decided to report it with a review of literature. A 70-year-old man was informed of a liver tumor by his previous doctor and referred to us for further examination and treatment. Contrast-enhanced CT revealed a 3.5 cm cyst in the S2/3 of the liver and a 3.2 cm tumor with an early staining within the cyst. Laparoscopic lateral segmentectomy was performed and a diagnosis of hepatic cystic adenocarcinoma was made. Macroscopically, the tumor was white and welldefined. Histopathological examination revealed round chromatin-enriched tumor cells proliferated with round fibrillar cells or intercalated structures consisting of round or short spindle-shaped cells. Immunohistochemistry yielded negative results for CAM5.2, HepPar1, CD31, CD99, SMA, and HMB45 but positive results for CD34, Factor Ⅷ, Bcl-2, and STAT6; therefore, the patient was further diagnosed with liver SFT. Although liver SFT is rare, it was considered a differential diagnosis when multiple bloody liver tumors were detected. In addition, since there were reports of recurrence, careful follow-up in future, was deemed necessary.

Cerebral arterial air emboli after stent insertion in esophageal cancer complicated with esophago-left atrial fistula: An autopsy case and review of the literature.

Cerebral arterial air embolism is a rare and unexpected complication of advanced esophageal cancer. The entry of air to systemic circulation is an esophago-left atrial or pulmonary vein fistula formation. Herein, we report an autopsy case of a 64-year-old man. He was diagnosed esophageal cancer 2 years ago and underwent chemotherapy and concurrent chemoradiotherapy but the disease progressed, unfortunately. Then two metal stents were inserted into the middle thoracic esophagus as a palliation of dysphagia. After initiation of oral intake, he developed deterioration of consciousness. The cranial computed tomography showed cerebral arterial air emboli with multiple low-density areas. He failed to gain consciousness again and died one and half days later. In a literature survey, this autopsy case is the first presentation that confirmed histologically the close association between stent placement and formation of esophago-left atrial fistula. Due to the fatality of cerebral arterial air embolism, clinicians should keep in mind the possibility of this catastrophic complication after multimodality treatment of esophageal cancer.

[A Case of Stage IV Metaplastic Breast Cancer in Which Primary Tumor Was Removed after Treatment with Anti-HER2 Systemic Therapy].

The patient was a 58-year-oldpostmenopausal woman. Vacuum assistedbiopsy of the left breast tumor revealedinvasive ductal carcinoma. Immunohistochemical examination was negative for estrogen receptor(ER), negative for progesterone receptor(PgR), andshowedan HER2 score of 3+. FDG-PET/CT revealedmultiple metastases to the left supracravicular and axillary lymph nodes and lungs. She was diagnosed with HER2-positive T3N3M1, Stage IV breast cancer. A 2-year regimen of chemotherapy with trastuzumab andvinorelbine achieveda complete response with regardto the metastatic sites; however, the size of the primary tumor increasedd espite the chemotherapy, andsurgical resection of the left breast with axillary lymph node dissection was performed for local control. Pathological examination of the surgical specimen revealed metaplastic carcinoma with sarcoma component surrounded by non-invasive ductal carcinoma. No component of invasive ductal carcinoma was found. Immunohistochemically, metaplastic carcinoma was negative for ER, negative for PgR, andrevealedan HER2 score of 0. There was discordance of HER2 status between pre- andpost -chemotherapy. The patient receivedno further chemotherapy following surgery andhas been without disease progression for 6 years. We suggest there is heterogeneity, that is, the metastatic sites andthe partial primary tumor were HER2-positive invasive ductal carcinoma and the remainder of the primary tumor was triple negative metaplastic carcinoma. As a result, the patient was able to discontinue chemotherapy with higher quality of life.

[A Case of Local Recurrence after Conserving Therapy for Occult Breast Cancer with Immediate Breast Reconstruction].

We report the case of a 70-year-oldwoman with local recurrence of occult breast cancer after conserving therapy. Breast cancer metastasis to an axillary lymph node was suspected when she was 62-years-old. Even with mammography, ultrasound sonography, positron emission tomography/computedtomography, andmagnetic resonance imaging, the primary disease lesion could not be identified. She underwent axillary dissection, and received 5 years of endocrine therapy. Eight years after surgery, a new ipsilateral breast tumor was detectedusing ultrasoundsonography andvacuum assistedbiopsy, confirmedas invasive carcinoma. This time the patient had no distant metastases; therefore, she was diagnosed with local recurrence, and mastectomy with immediate breast reconstruction was performed. Breast conserving surgery aims to reduce the psychological burden on the patient but must be closely followed-up in case of local recurrence. Any incidences of local recurrence can also be treatedusing breast reconstruction surgery.

[A Case of Pure Squamous Cell Carcinoma of the Breast in an Elderly Woman Diagnosed by Cytology].

A n 85-year-old woman presented with a mass in the left breast. A3 7mm lobulated mass including enhancement of a cyst of 37mm was detected by sonography. An axillary lymph node had increased to 16 mm. Atypical cells dyed by light green and orange G were identified by fine needle aspiration and cytology. She was diagnosed with left breast cancer(cT2N1M0, Stage II B), histologically suspected to be squamous cell carcinoma. She underwent a left-breast-conserving surgery and axillary lymph node dissection. On pathology, a cyst of 34×30mm was noted. The tumor grew from inside the cyst to the surround- ing tissue and it had a trend for keratinocytes. Lymph node metastases affected 1/11. By immunostaining, the tumor was found to be ER(+), PgR(-), HER2(-), CK5/6(+), p40(+), mammaglobin(-), and GCDFP15(-). There was no component of ductal carcinoma. She received radiotherapy to the left breast and tamoxifen as an adjuvant therapy. Squamous cell carcinoma of the breast is rare. We encountered a case of pure squamous cell carcinoma of the breast in an elderly woman. We report this case with a discussion of the relevant literature.

[A Case of Peritoneal Metastasis of Breast Cancer Diagnosed by Laparoscopic-Assisted Right Hemicolectomy].

The patient was an 86-year-old woman. She underwent right breast-conserving surgery and sentinel lymph node biopsy for breast cancer in August 2006. The pathological diagnosis was invasive ductal carcinoma, T1N0M0, Stage Ⅰ, ER (+), PgR (-), HER2 (-). She was treated with tamoxifen for 5 years as adjuvant therapy and showed no signs of recurrence. In November 2014, CA15-3 was elevated and an accumulation of FDG in the right paracolic sulcus was observed on PET-CT. Peritoneal metastasis of breast cancer was suspected, and an operation was performed for a definitive diagnosis. During the operation, the tumor was seen on the paracolic sulcus, and laparoscopic-assisted right hemicolectomy was performed. A poorly differentiated adenocarcinoma was diagnosed by pathological examination, and immunostaining results were as follows: CK7(+), CK20(-), mammaglobin (-), GCDFP-15 (-), ER (-), PgR (-), and HER2 (-). Because there was no original lesion other than the breast cancer, the tumor was diagnosed as a metastasis of breast cancer. The frequency of peritoneal metastasis of breast cancer is low. In this case, pathological diagnosis was necessary for a definitive diagnosis. A change of subtype was also confirmed, and the treatment strategy was decided appropriately. Surgical resection should be considered for peritoneal metastasis of breast cancer when the operation can be performed safely.

[A case of hyoid bone metastasis from breast cancer].

A 69-year-old woman had undergone breast conserving surgery and axillary lymph node dissection for left breast cancer 10 years previously. The tumor was positive for estrogen receptor (ER) and progesterone receptor (PgR), and negative for human epidermal growth factor receptor-2 (HER2). Adjuvant tamoxifen and radiation therapy were administered to the conserved breast for 5 years. The patient detected a painless neck mass 1 year previously. Computed tomography (CT) revealed a hyoid bone mass, and fine needle aspiration cytology indicated a diagnosis of adenocarcinoma. Positron emission tomography combined with CT (PET-CT) revealed masses in the pelvis, spine, hyoid bone, and cervical lymph node. For definitive diagnosis, excisional biopsy of the hyoid bone was performed. Immunohistostaining revealed that the cells were CK7 (+), CK20(-), mammaglobin (+), GCDFP-15 (+), ER (+), PgR (+), and HER2 (-). The final diagnosis was multiple bone metastasis(hyoid, pelvis, spine)as well as cervical lymph node metastasis from breast cancer. After diagnosis, the patient was treated with anastrozole and denosumab, and she achieved a partial response. She has experienced progression free survival for 12 months. Metastasis to the hyoid bone is uncommon for breast cancer. We report a case in which hormone therapy was effective after it was selected based on the results of excisional biopsy.

[A case of malignant phyllodes tumor after surgery for breast cancer].

A 63-year-old woman underwent breast-conserving resection and axillary lymph node dissection for bilateral breast cancer in December 2008. Histopathological diagnosis for the right breast cancer was t=1.3 cm, n=1/29, estrogen receptor (ER) (+), progesterone receptor (PgR) (+), human epidermal growth factor receptor 2(HER2) (-), and that for the left breast cancer was t=1.8 cm, n=9/28, ER (+), PgR (+), and HER2 (-). She was administered adjuvant chemotherapy (4 courses of fluorouracil, epirubicin, and cyclophosphamide [FEC 100] and 4 courses of docetaxel[DTX], 75 mg/m²), letrozole, and bilateral radiation therapy for the remaining breast tissue. She noticed a mass in the left breast in December 2013. Fine-needle aspiration cytology and core-needle biopsy indicated a malignant phyllodes tumor or stromal sarcoma. Positron emission tomography-computed tomography (PET-CT) revealed the accumulation of fluorodeoxyglucose (FDG) only in the mass. The tumor enlarged rapidly to more than 5 cm during the pre-operative period. In January 2014, the patient underwent left mastectomy. The histopathological diagnosis was malignant phyllodes tumor in the left breast, with a tumor diameter of 7 cm, and negative margins. Presently, 6 months after the operation, the patient is alive without recurrence.

[A case report of matrix-producing carcinoma of the breast].

A 43 -year-old woman with a palpable mass in the right breast consulted a neighborhood doctor. She was diagnosed with right breast cancer after core needle biopsy, and she was referred to our hospital. Mammography revealed an indistinct mass with calcification in the lower outer quadrant of the right breast. Ultrasonography revealed a hypoechoic mass with a high echo spot. Magnetic resonance imaging (MRI) revealed a high intensity tumor with peripheral enhancement. The patient underwent mastectomy with sentinel lymph node(SN) biopsy and axillary lymph node dissection. Histologically, the tumor was composed of a solid-tubular carcinoma with a centrally located metaplastic cartilaginous element. There was an abrupt transition between these components, without intervening spindle cells or osteoclastic cells. Subtyping via immunohistochemical analysis for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth factor receptor 2 (HER2) demonstrated that the tumor was triple negative (TN). The histological diagnosis was matrix-producing carcinoma (MPC). Adjuvant chemotherapy was administered, and she has been recurrence-free. MPC has unique features, such as emphasis of the peripheral zone of the tumor by using contrast enhanced-computed tomography (CT) and gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) MRI. Most cases of MPC that have been reported were TN. The 5 year survival rate for MPC is poorer than that for breast cancer. In Japan, 7 cases of MPC recurrence have been reported within 2.5 years, suggesting that careful follow-up is necessary for 2-3 years after surgery.

[Identification of the primary lesion in a patient with concomitant breast and kidney cancer following fracture of the femur].

A 61-year-old woman was diagnosed with breast cancer [T3N3cM0: Stage IIIC, estrogen receptor [ER] (+), progesterone receptor [PgR] (+), human epidermal growth factor receptor 2[HER2] (-)]at the time of initial presentation. Following diagnosis, combined modality therapy including hormone therapy and chemotherapy were initiated, but hemorrhage from the primary lesion and bone metastases were observed. Priority was given to treatment of the breast cancer, and chemotherapy was administered, after which, right mastectomy and axillary lymph node sampling were performed to assess local disease control. In addition, concurrent right kidney enucleation was performed for a renal lesion. The renal neoplasm was diagnosed as T1aN0M0, Stage I. After this intervention, treatment of the breast cancer was continued, but pain of the right femoral region developed, and bone metastasis was diagnosed on close inspection. The bone metastasis was considered to derive from the breast cancer. During hospitalization, the patient fell and broke her right femur. Open reduction and internal fixation was performed immediately, and bone metastasis of kidney cancer was diagnosed via perioperative cytodiagnosis. Pulmonary metastasis, local recurrence, and metastasis to the shoulder blade have been detected. The metastases are considered to derive from the breast cancer, for which treatment has been continued. In the case of concomitant cancers, biopsy for metastatic foci can be considered essential, whenever it can be performed safely.

Self-limited effusion large B-cell lymphoma: two cases of effusion lymphoma maintaining remission after drainage alone.

We report two cases of human herpesvirus-8 (HHV-8)-negative large B-cell lymphoma involving pericardial and/or pleural effusion that regressed after drainage alone. Case 1 is a 70-year-old man showing massive pericardial effusion. Cytology of the drained effusion showed monotonous infiltration of CD3-, CD20+, CD79a+, and CD138- large B-cells. Monoclonality was shown by Southern blot analysis. Case 2 is a 70-year-old man with massive pericardial and bilateral pleural effusion. Cytology of pericardial effusion showed infiltration of CD20+, CD45RO-, CD138-, immunoglobulin lambda chain+, and kappa chain- large B cells. In both cases, effusion resolved after drainage and no relapse has been observed. HHV-8 was not demonstrated in either case. Clinical presentation of our two cases resembled primary effusion lymphoma (PEL), but cytomorphology, immunophenotype, and prognosis were clearly distinct from those of PEL. HHV-8-negative effusion lymphomas might include prognostically favorable self-limited tumors that could regress without any cytotoxic therapy.

[Is standard radical mastectomy still a treatment option for select patients with breast cancer ?].

BACKGROUND: Currently, minimal surgery is the treatment of choice for most breast cancer patients. However, some patients with locally advanced breast cancer( LABC) require standard radical mastectomy( SRM) before or after systemic therapy. PATIENTS AND METHODS: We reviewed the medical records of 7 patients with breast cancer who underwent SRM in our hospital between January 2007 and April 2013 and examined their clinicopathological features and prognostic outcomes. RESULTS: The average patient age was 68 years. SRM was performed because of muscle invasion( n=6), Rotter lymph node metastases( n=3), and level III lymph node metastases( n=3). After SRM, the wound was closed by skin grafting in 2 of the 7 patients. With regard to postoperative complications, 2 patients required rehabilitation for shoulder joint stiffness, whereas wound infection and arm edema were encountered in 1 and 2 patients, respectively. During the median follow-up time of 26 (range; 4-69) months, 3 patients experienced recurrence. The metastatic sites in the 3 patients were the supraclavicular lymph nodes, the chest wall, and the liver, respectively. CONCLUSIONS: Quality of life( QOL) following SRM was acceptable in the patients examined. SRM could be considered as a treatment option in select patients with LABC.

A case of clear cell adenocarcinoma arising from the urethral diverticulum: Utility of urinary cytology and immunohistochemistry.

Carcinomas rarely arise from the urethral diverticulum. In this report, we present a case of clear cell adenocarcinoma arising from the urethral diverticulum. A 42-year-old woman complained of bloody discharge and lower back pain. Imaging studies showed a tumor involving the region surrounding the urethra and cystourethroscopy showed papillary and villous tumors in the urethral diverticula. Cytology of the urine sediment showed papillary or spherical clusters of atypical cells, some of which had clear abundant cytoplasm and formed mirror ball-like clusters, suggesting adenocarcinoma. Although histological diagnosis was indeterminate by biopsy and transurethral resection (TUR) because of absence of stromal invasion, surgically resected specimen via cysturethrectomy revealed that the tumor was clear cell carcinoma. Urinary cytological findings and immunohistochemical analysis for CD15, Ki-67, and p53 might be useful for accurate diagnosis of clear cell adenocarcinoma that arises from the urethral diverticulum when sufficient materials are not available by biopsy and TUR.

Mucinous borderline-like tumor of the gastrointestinal type arising from mature cystic teratoma of the ovary and its immunohistochemical cytokeratin and mucin phenotype.

Malignant transformation is rarely seen in the disease course of mature cystic teratoma (MCT) of the ovary. Adenocarcinoma arising from MCT is especially rare. We herein present the case of a premenopausal woman with a mucinous borderline-like tumor arising from a MCT. Based on the histological transition between the borderline-like tumor and gastrointestinal elements of the MCT, we consider that the tumor derived from teratomatous gastrointestinal epithelium. Immunohistochemistry showed that the proliferating mucinous cells were diffusely positive for cytokeratin 20 and partially positive for cytokeratin 7. MUC5AC was partially positive, whereas MUC2 and MUC6 were positive in a small number of tumor cells. The immunophenotype of cytokeratins and mucins in the present case was compatible with malignant transformation of the teratomatous gastrointestinal epithelium.

A large retroperitoneal cystic venous malformation mimicking bilateral ovarian cystic tumors.

INTRODUCTION: Retroperitoneal cysts are a rare disease. Most retroperitoneal cysts of vascular origin have been reported as hemangiomas. However, according to the recent classification of vascular anomalies accepted by the International Society for Study of Vascular Anomalies (ISSVA), these previously reported retroperitoneal hemangiomas should rather have been classified as vascular malformations. CASE REPORT: A 65-year-old woman visited our hospital complaining of a sense of unexplained abdominal fullness. Magnetic resonance imaging suggested a uterine leiomyoma and bilateral ovarian cystic tumors. However, abdominal surgery revealed normal bilateral ovaries, but huge cystic masses in the retroperitoneum. Postoperative histological diagnosis of the retroperitoneal cysts demonstrated that they were venous malformations. CONCLUSION: This is a rare case in which large cystic retroperitoneal venous malformations were preoperatively diagnosed as ovarian cystic tumors. Retroperitoneal hemangiomas should be renamed as vascular malformations following the ISSVA classification.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type arising in the pleura with pleural fibrous plaques in a lathe worker.

Our patient was an 86-year-old man who had worked as a lathe operator for 40 years. He had no history of tuberculosis, pyothorax, or autoimmune disease. He had not been exposed to asbestos. He was asymptomatic, but an imaging study showed gradually increasing pleural plaques. A biopsy specimen of a pleural lesion showed sclerosis of the pleura and diffuse infiltration of small- to medium-sized B lymphocytes. Polymerase chain reaction-based analysis detected monoclonal rearrangement of immunoglobulin heavy-chain genes. Histologic diagnosis was extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma). The lymphoma was negative for Epstein-Barr virus. We report a rare case of a metal worker with MALT lymphoma arising in the pleura with pleural fibrous plaques. It is speculated that MALT lymphoma might develop in the background of pneumoconiosis. Inflammatory and/or immunologic reactions to metal particles might contribute to the oncogenesis of this tumor.

[A case of primary neuroendocrine carcinoma of the bile duct].

A 74-year-old man presented to a physician with a chief complaint of jaundice. He was diagnosed with bile duct carcinoma and admitted to our hospital. Laboratory data revealed abnormally elevated levels of total bilirubin, serum hepatic transaminase, and CA19-9. Endoscopic retrograde cholangiopancreatography revealed neoplastic stenosis from the hilus hepatis to the common bile duct. Abdominal computed tomography (CT) revealed an enhancing tumor in the hilus hepatis bile duct, and positron emission tomography-CT (PET-CT) revealed abnormal fluorodeoxyglucose accumulation in the tumor. Under a diagnosis of hilar cholangiocarcinoma, the patient underwent an extended right hepatectomy and left hepatico -jejunostomy. Immunohistochemically, the tumor cells were positive for neuroendocrine markers such as chromogranin A, synaptophysin, and CD56. The tumor was diagnosed as primary neuroendocrine carcinoma of the bile duct. The patient exhibited multiple liver metastasis 6 months after the operation. Transcatheter arterial chemoembolization (TACE) was performed for the liver metastasis. Although TACE exerted a cytoreductive effect temporarily, multiple liver abscesses developed. The patient died of liver failure 16 months after the operation. We report this rare case of primary neuroendocrine carcinoma of the bile duct.

Synchronous primary malignancies in ovarian cancer and liver angiosarcoma.

•A case of concurrent primary ovarian clear cell adenocarcinoma and liver angiosarcoma is detailed herein.•If a liver tumor is found together with ovarian cancer, it is necessary to determine whether this is a primary hepatic malignancy or metastatic liver cancer.•It is important to make a definitive diagnosis by performing a liver biopsy when appropriate.

Pleural MALT lymphoma diagnosed on thoracoscopic resection under local anesthesia using an insulation-tipped diathermic knife.

A 79-year-old man presented with back pain. Chest CT scan showed elevated nodular lesions in the right parietal pleurae with pleural effusion. There were no intrapulmonary or mediastinal abnormalities. Under local anesthesia, right thoracoscopy and subsequent thoracoscopic pleural resection were performed using an insulation-tipped diathermic knife (IT-knife). The resected pleura, 2.2 cm in diameter, had a rough granular surface. Lymphoid cells histologically infiltrated diffusely into the pleura. They were composed of centrocyte-like and monocytoid cells. On immunohistochemistry they were found to be positive for Bcl2, CD20, CD45RB and CD79a, but negative for CD3, CD5, CD10 and cyclin D1. EBV-encoded small RNA-1 (EBER-1) in situ hybridization was negative. A diagnosis of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) arising in the pleura was therefore made. To the authors' knowledge this is the first case in which IT-knife was used for diagnosis of a pleural lesion. This large, single-piece, only slightly crushed pleural specimen, enabled study of histopathological findings (listed here) that could not have been obtained on conventional biopsy: (i) lack of apparent evidence of plasmacytic differentiation; (ii) no recognition of lymphoid follicles; (iii) mesothelial cells not infiltrated by lymphoma cell clusters; (iv) thin layer of hyperplastic mesothelial cells continuously covering the surface; and (v) no proliferation of fibroblast-like submesothelial cells.