Cancer-related thrombotic microangiopathy and disseminated intravascular coagulation in a patient with bone marrow carcinomatosis of unknown primary origin: A case report.

BACKGROUND: Cancer-related thrombotic microangiopathy (CR-TMA) is a rare type of Coombs-negative hemolytic anemia, which is caused by malignancy and has a poor prognosis. CASE: A 76-year-old female was referred to our hospital due to Coombs-negative hemolytic anemia, which was causing fatigue and dyspnea on exertion, accompanied by schistocytosis. A bone marrow examination demonstrated bone marrow carcinomatosis, and the tumor cells were morphologically suspected to be signet-ring cell carcinoma cells. As we failed to find the primary tumor site before the patient died, she was diagnosed with CR-TMA due to bone marrow carcinomatosis of unknown primary origin. Thrombotic thrombocytopenic purpura (TTP) was rapidly ruled out based on her PLASMIC score. In addition, immunohistochemical staining of a clot section of the bone marrow and tumor marker data were useful for narrowing down the likely primary tumor site. CONCLUSION: Although CR-TMA is an extremely rare phenomenon, clinicians who suspect CR-TMA should quickly rule out TTP and decide whether to provide appropriate chemotherapy or plan for palliative care.

[Onset of Testicular Plasmacytoma in a Patient with Refractory Multiple Myeloma].

A 72-year-old male patient, who had been on chemotherapy for the treatment of IgG-λ multiple myeloma, presented an enlargement of the testis 3 years and 5 months after the diagnosis. High orchiectomy was then performed, leading to the diagnosis of plasmacytoma. Due to residual disease, treatment with a combination of isatuximab and dexamethasone was initiated. The patient is currently under follow-up without recurrence. While testicular tumors are difficult to diagnose by imaging studies alone and extramedullary plasmacytomas rarely occur in the testis, pathological assessment is critical for treatment planning.

[Primary Diffuse Large B-Cell Lymphoma of the Testis Developed during the Course of Mycosis Fungoides-A Case Report].

We report the case of an 84-year-old man who developed primary diffuse large B-cell lymphoma of the testes during the course of mycosis fungoides treated with topical medication. He was referred to our hospital due to bilateral testicular masses, and bilateral high orchiectomy was performed. A pathological diagnosis of diffuse large B-cell lymphoma was made after an examination of the surgical specimen. Rituximab-combined miniCHOP chemotherapy with prophylactic intrathecal injection resulted in complete remission without recurrence 1 year after diagnosis. People with mycosis fungoides are known to be at a higher risk of secondary malignancies than healthy individuals; hence, a pathological examination is important to confirm the diagnosis.

[A Case of Spindle Cell Tumor in the Gastric Mucosa Treated with Laparoscopic Local Gastrectomy].

The patient was a 43-year-old man. An upper gastrointestinal endoscopic examination revealed a gastric submucosal tumor(SMT)-like, elevated 8-mm lesion in the greater curvature of the upper body of the stomach. It was diagnosed as spindle cell tumor on the basis of biopsy findings, and a gastrointestinal stromal tumor(GIST)was suspected. Various immunohistochemical staining techniques were used; however, a definitive diagnosis could not be achieved. There was no evidence of distant metastasis even on thoracoabdominal computed tomography imaging; thus, the patient was referred to our department for definitive diagnosis and surgical treatment. Laparoscopic local gastrectomy with concomitant intraoperative gastroscopy was performed. Pathological examination of the resected specimen showed a type Ⅱc-like lesion with a maximum diameter of 6 mm in the mucosal layer along with spindle cell proliferation. Immunostaining was negative for c- kit, DOG1, CD34, S-100, SMA, WT-1, desmin(N), EMA, and keratin(pan)and positive for ß-catenin, Bcl-2, and vimentin; furthermore, low Ki-67(MIB-1)expression was detected. Therefore, GIST, solitary fibrous tumor, leiomyoma, leiomyosarcoma, desmoid tumor, spindle cell carcinoma, and synovial sarcoma were excluded, and an unclassifiable spindle cell tumor arising from the gastric mucosa was diagnosed. The patient has remained recurrence-free for 1 year and 8 months post-operatively and is currently under careful outpatient follow-up.

[A Case of Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater].

An 80-year-old women admitted to our hospital with jaundice. Abdominal contrast-enhanced CT scan revealed an enhanced tumor, measuring 10 mm, at the duodenal ampulla. Upper endoscopy showed a submucosal tumor-like lesion at the duodenal ampulla. Immunohistochemical findings showed positive for chromogranin A and synaptophysin, and neuroendocrine carcinoma was diagnosed. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed. The final diagnosis was large cell neuroendocrine carcinoma(LCNEC). Multiple metastases of liver, lung and bone were occurred 14 months after the surgery, and she died 21 months after the surgery. LCNEC of the duodenal ampulla is very rare, and its prognosis is poor.

[A Case of Omental Desmoid Tumor after a Small Bowel Resection for Gastrointestinal Stromal Tumor].

An intra-abdominaldesmoid tumor, especially omentaldesmoid tumor, is rare. Here, we report a case of omentaldesmoid tumor after a smallbowelresection for gastrointestinalstromaltumor (GIST). A 73-year-old man underwent a partial resection of smallbowelfor GIST. He received adjuvant therapy with imatinib due to high risk of recurrence. After 2.5 years of treatment, a follow-up CT showed a 15mm nodule in the omentum near the splenic flexure. We considered the possibility of recurrence and imatinib failure, and laparoscopic tumor resection was performed for differential diagnosis. Immunohistochemicalstaining showed negative for c-kit, CD34, desmin, and S100. However, it was diagnosed as desmoid tumor because of positive b-catenin. Intra-abdominal desmoid tumor should be a differential diagnosis for a new single lesion in patients with GIST.

An isolated der(1;21)(q10;q10) translocation in a patient with myelodysplastic syndrome: a case report.

Whole-arm translocations are relatively rare among hematological malignancies. There are a few reports on myeloid malignancies harboring der(1;21)(q10;q10). A 65-year-old male was referred to our hospital due to squamous cell carcinoma of the lung. Pembrolizumab monotherapy resulted in progression, and so chemotherapy involving nab-paclitaxel and carboplatin was administered thereafter. The patient developed cytopenia, and his bone marrow exhibited dysplasia. Chromosomal analysis revealed a whole-arm translocation, der(1;21)(q10;q10). Thus, the patient was diagnosed with myelodysplastic syndrome. The der(1;21)(q10;q10) translocation is a rare variant of the der(1;7)(q10;p10) translocation, which is an adverse prognostic factor for myeloid neoplasms. Clarifying the clinical features of myeloid neoplasms in patients with der(1;21)(q10;q10) would facilitate the elucidation of their tumorigenic mechanisms.

Trisomy 6 as the sole stemline abnormality in a patient with acute monocytic leukemia: a case report.

It is rare for trisomy 6 to occur as the sole autosomal anomaly in hematological malignancies, but this finding has been reported to be associated with a hypoplastic bone marrow. We report the case of a 75-year-old male with acute monocytic leukemia, in which trisomy 6 was detected as the sole stemline abnormality. We also summarize the 26 published cases of acute myeloid leukemia involving isolated trisomy 6.

[Long-Term Disease-Free Survival by Curative Resection in a Case of Remnant Gastric Cancer with Peritoneal Recurrence].

The patient was 69-year-old man. For the remnant gastric cancer, partial resection of the remnant stomach with combined resection of mesentery of transverse colon was performed. Pathological diagnosis was adenocarcinoma(tub2>tub1), M, B- 50-AJ, type 3, pT4b(mesentery of transverse colon), pN0, CY0. A CT scan of 6 months after the surgery showed a tumor on the left side abdomen and diagnosed as peritoneal recurrence. Chemotherapy consisted of 1 course of TS-1(100mg/body) plus cisplatin(70mg/body), 4 courses(2 weeks administration and 1 week break)of TS-1(100mg/body), 8 courses of docetaxel(80mg/body). Tumor shrinkage and internal necrosis were observed. Peritoneal tumor was resected 19 months after the first surgery, and partial resection of the invaded transverse colon and jejunum was performed. Pathological diagnosis was metastasis of remnant gastric cancer. After that, it is 61 months since the first surgery and 42 months from the recurrence surgery without relapse.

L-menthol improves adenoma detection rate during colonoscopy: a randomized trial.

BACKGROUND AND STUDY AIMS: Colonoscopy is one of the most reliable methods for the detection of colorectal neoplasms. However, colonic peristalsis during colonoscopy results in some neoplastic lesions being hidden from view and commonly requires an intravenous or intramuscular injection of antispasmodic agents, which may sometimes causes unexpected adverse reactions. The aim of this study was to evaluate the efficacy of L-menthol spray as an antiperistaltic agent and its effect on adenoma detection. PATIENTS AND METHODS: This was a prospective, randomized, single-blind placebo-controlled trial. A total of 226 patients who were scheduled to undergo colonoscopy were randomly assigned to receive either 20 mL of 1.6 % L-menthol (n = 118) or placebo (n = 108). Both treatments were sprayed locally onto the colonic mucosa via an endoscope. The adenoma detection rate (ADR) and the proportion of patients with no peristalsis were the primary and secondary outcomes, respectively. RESULTS: The ADR was significantly higher in the L-menthol group than in the placebo group (60.2 % vs. 42.6 %; P = 0.0083). The proportion of patients with no peristalsis after treatment with L-menthol was significantly higher than in the placebo group (71.2 % vs. 30.9 %; P < 0.0001). There were no adverse effects in either group. CONCLUSIONS: The results suggest that the suppression of colonic peristalsis by L-menthol sprayed directly onto the colonic mucosa improves the ADR. CLINICAL TRIAL REGISTRATION: ID: UMIN 000007972.

Anatomical liver segmentectomy 2 for combined hepatocellular carcinoma and cholangiocarcinoma with tumor thrombus in segment 2 portal branch.

BACKGROUND: Hepatic resection is the only effective treatment for combined hepatocellular carcinoma and cholangiocarcinoma. CASE PRESENTATION: A 52-year-old man was preoperatively diagnosed with hepatocellular carcinoma in segment 2 with tumor thrombus in the segment 2 portal branch. Anatomical liver segmentectomy 2, including separation of the hepatic arteries, portal veins, and bile duct, enabled us to remove the tumor and portal thrombus completely. Modified selective hepatic vascular exclusion, which combines extrahepatic control of the left and middle hepatic veins with occlusion of left hemihepatic inflow, was used to reduce blood loss. A pathological examination revealed combined hepatocellular carcinoma and cholangiocarcinoma with tumor thrombus in the segment 2 portal branch. No postoperative liver failure occurred, and remnant liver function was adequate. CONCLUSION: The separation method of the hepatic arteries, portal veins, and bile duct is safe and feasible for a liver cancer patient with portal vein tumor thrombus. Modified selective hepatic vascular exclusion was useful to control bleeding during liver transection. Anatomical liver segmentectomy 2 using these procedures should be considered for a patient with a liver tumor located at segment 2 arising from a damaged liver.

A Case of Neuroendocrine Cell Carcinoma with Sigmoidovesical Fistula.

Colonic neuroendocrine cell carcinoma (NEC), which is a rare subtype of colon epithelial neoplasm, has been reported to show extremely aggressive characteristics with a 1-year survival rate of 20%. We report herein a resected case of NEC that manifested bacterial sepsis due to sigmoidovesical fistula. Staged surgery consisted of resecting the sigmoid colon and part of the bladder four weeks after construction of an ileostomy to alleviate septic shock. The resected specimen was histologically diagnosed as NEC invading the wall of the urinary bladder with metastasis to the regional lymph nodes. The patient underwent four cycles of FOLFOX after surgery for additional treatment of residual metastatic lymph nodes around the abdominal aorta diagnosed preoperatively. Although the patient showed stable disease measured by computed tomography scan for the first three months after surgery, he rejected additional chemotherapy thereafter, and died ten months after the initial admission due to progression of residual tumor in the urinary bladder as well as the lymph nodes. This is the first case report describing colonic NEC manifesting perforation into the urinary bladder. Although the optimal chemotherapeutic regimen for colonic NEC has not yet been established, FOLFOX may be one of the choices.

Multifocal intraportal invasion of breast carcinoma diagnosed by laparoscopy-assisted liver biopsy.

Hepar lobatum carcinomatosum (HLC) is defined as an acquired hepatic deformity consisting of an irregularly lobulated hepatic contour caused by intravascular infiltration of metastatic carcinoma. To date, only nine cases of HLC have been reported in the literature. We report a case of a 68-year-old woman showing hepatic metastasis of breast carcinoma in radiologically unidentified form. Initially, she received left partial mastectomy for breast cancer but solid hepatic metastases were identified in S(2) and S(6), 9 mo after surgery. Then, they responded to chemotherapy and radiologically disappeared. After radiological disappearance of the liver tumors, the patient's blood chemistry showed abnormal liver function. A CT scan demonstrated heterogeneous enhancement effect in the liver in the late phase, suggesting uneven hepatic blood supply. Hepatic deformity was not obvious. Laparoscopy revealed a slightly deformed liver surface with multiple indentations and shallow linear depressions. Furthermore, a wide scar was observed on the surface of S(2) possibly at the site where the metastatic tumor existed before chemotherapy. Liver biopsy from the wide scar lesion showed intraportal tumor thrombi with desmoplastic change. Because of its similarity to the histology of the original breast cancer, we concluded that the hepatic functional abnormalities and slightly deformed liver surface were derived from the circulatory disturbance caused by microscopic tumor thrombi. Besides, since the wide scar was located at the site of the pre-existing tumor, it is probable that chemotherapy was an important cause of fibrous scarring as a result of tumor regression. These morphologic findings are compatible with those of HLC. Laparoscopy-assisted liver biopsy was useful to make definite diagnosis, even though the hepatic deformity was radiologically undetectable.

Irritated seborrheic keratosis of the external ear canal.

Although a seborrheic keratosis is not rare on the skin of the trunk, extremities, head and neck of an elderly person, it is uncommon to originate in the external ear canal. Even rarer, an irritated subtype may arise in this location. We report the case of a 63-year-old man with an irritated seborrheic keratosis arising in the right external ear canal, who first presented with a bloody discharge from his right ear canal 3 years and 10 months before the subsequent operation. Macroscopically, a dome-like, black elevation was found at the anterior wall of the right external ear canal. A biopsy showed an exophytic papillomatous, hyperkeratotic growth of basaloid cells which revealed some nuclear atypism and mitosis. Squamous eddies and moderate inflammatory infiltrate were noted. Some cellular atypism made us suspect an irritated seborrheic keratosis. Because the patient hated the extirpation, he remained untreated for 3 years and 3 months following his first presentation. The biopsy and a surgical resection of his second presentation confirmed the diagnosis of irritated seborrheic keratosis. Immunohistochemical analysis of Ki-67, p53 and papillomavirus was performed to elucidate its character. We emphasize that irritated seborrheic keratosis does occur in the external ear canal and may cause some diagnostic difficulty. Pathologists should be aware of this fact and avoid overdiagnosis.

Cytologic features of atypical polypoid adenomyoma of the endometrium. A case report.

BACKGROUND: In 1981, Mazur reported the histologic characteristics of atypical polypoid adenomyoma (APA) of the endometrium. Although most APAs of the endometrium are considered to show benign behavior, there is a small associated risk of the development of adenocarcinoma. The histology of APA of the endometrium is well defined, but the cytologic features of the lesion have not yet been clarified. CASE: A 28-year-old nulligravida with hypermenorrhea had an exophytic, polypoid mass arising from the posterior uterine wall on ultrasonography and magnetic resonance imaging. The results of endometrial smear and biopsy were normal. Transcervical total resection of the tumor was performed with a resectoscope. Frozen sections of the sample suggested APA of the endometrium, and the permanent sections confirmed the diagnosis. The tumor stump/resection plane smears revealed overlapping, highly atypical glandular cells with enlarged, hyperchromatic nuclei; squamous metaplastic cells; and abundant, spindled smooth muscle cells on a clear background, effectively reflecting the epithelial and mesenchymal cell components of the lesion. CONCLUSION: Endometrial smear and biopsy are inaccurate methods for the diagnosis of APA of the endometrium because of limited sampling. Tumor stump/resection plane cytology appears to be useful for detecting APA of the endometrium.