Extraordinary Response of Metastatic Pancreatic Cancer to Chemotherapy / 대한소화기암학회지

A 58-year-old woman presented with right flank and back pain for one month. After undergoing an abdominal computed tomography (CT), she was referred to our hospital. The abdominal CT showed a hypodense pancreatic tail mass with multiple retroperitoneal lymph node metastases. Positron emission tomography-computed tomography (PET-CT) scan showed high 18F-FDG uptake in pancreatic tumor and enlarged lymph nodes. Endoscopic ultrasound fine needle aspiration (EUS-FNA) revealed adenocarcinoma, which stained strongly in hENT1 (human equilibrative nucleoside transporter 1) on immunohistochemistry. She received gemcitabine 1,000 mg/m² + nanoparticle albumin-bound paclitaxel 125 mg/m² as a palliative chemotherapy. Follow-up abdominal CT and PET-CT after 4 cycles of chemotherapy showed that both pancreatic mass and the metastatic retroperitoneal lymph nodes were nearly disappeared. We report a case of 58-year-old female with metastatic pancreatic cancer who had a dramatic response to palliative chemotherapy (gemcitabine plus nanoparticle albumin-bound paclitaxel).

Pattern of hMLH1, hMSH2 and hMSH6 expression and clinical characteristics in a sample of Malaysian colorectal carcinoma cases

Malignant transformation from normal colonic mucosa to carcinomas may be accelerated by genetic loss or inactivation of genes of the DNA mismatch repair system. The aim of the study was to determine the local incidence and pattern of immunohistochemical expression of mismatch repair proteins namely: hMLH1, hMSH2 and hMSH6 in a series of colorectal carcinomas (CRCs) and correlate this to their clinical and pathological features. Forty-three out of 298 cases of CRCs (14.4%) showed abnormal staining pattern for mismatch repair proteins with a majority (65.1%) showing single hMLH1 loss. Tumours with mismatch repair defect (MMR-d) were frequently found at the right side of colon (p<0.001), poorly differentiated carcinomas (p<0.001), produced more mucin (p=0.007), exophytic growth (p=0.007) and were bigger (p=0.002) than tumours with no mismatch repair defect. Immunohistochemical stains for mismatch repair proteins could be done in local laboratories on these selected cases before referring for the expensive molecular test.

Cutaneous metastasis of prostate carcinoma to neck and upper chest.

Prostate adenocarcinoma is the most common urologic malignant neoplasm in men. Metastasis to skin is rarely reported and usually occurs late. The incidence and appearance of cutaneous metastasis are not well established in patients with prostate adenocarcinoma and their recognition remains poor among practicing urologists. Their clinical appearance may mimic other common dermatologic disorders. Definitive diagnosis requires a high index of suspicion. Immunohistochemical staining helps in establishing the diagnosis. We report a case of prostate adenocarcinoma presenting with widespread metastasis, including those to dermis and subcutaneous tissue of neck and upper chest.

Isolated primary schwannoma arising on the colon: report of two cases and review of the literature / 대한외과학회지

Primary schwannoma of the large intestine is an extremely rare neoplasm. Here, we report two cases of colonic schwannoma confirmed pathologically after laparoscopic resection. A 52-year-old female and a 59-year-old female were referred by their general practitioners to our coloproctologic clinic for further evaluation and management of colonic submucosal masses. Colonoscopies performed in our institution revealed round submucosal tumors with a smooth and intact mucosa in the mid-ascending and descending colon, respectively. Computed tomography (CT) scans showed an enhancing soft tissue mass measuring 2 x 2 cm in the right colon and well-defined soft tissue nodule measuring 1.5 x 1.7 cm in the proximal descending colon, respectively. We performed laparoscopic right hemicolectomy and segmental left colectomy under the preoperative impression of gastrointestinal stromal tumors. Two cases were both diagnosed to be benign schwannoma of the colon after immunohistochemical stains (S-100 (+), smooth muscle actin (-), CD117 (-), and CD34 (-)).

Predictive Value of the ERCC1 Expression for Treatment Response and Survival in Advanced Gastric Cancer Patients Receiving Cisplatin-based First-line Chemotherapy / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The aim of this study was to determine whether the ERCC1 expression is effective to predict the clinical outcomes of patients with advanced gastric cancer (AGC) and who were treated with cisplatin-based first-line chemotherapy. MATERIALS AND METHODS: A total of 89 measurable AGC patients received cisplatin and capecitabine, with or without epirubicin, as a part of a randomized phase II study. Patients were included for the current molecular analysis if they had received two or more cycles of chemotherapy, their objective tumor responses were measured and if their paraffin-embedded tumor samples were available. The ERCC1 expression was examined by performing immunohistochemical (IHC) staining, and the patients were divided into two groups (positive or negative) according to the presence of IHC staining of the tumor cell nuclei. RESULTS: Of the 32 eligible patients, 21 patients (66%) had tumor with a positive expression of ERCC1 and the remaining 11 patients had tumor with a negative ERCC1-expression. The ERCC1-negative patients achieved a higher response rate than that of the ERCC1-positive patients (44% vs. 28%, respectively), although the difference was not statistically significant (p=0.42). The median survival time for the all patients was 14.6 months (95% CI: 13.6 to 15.6 months). The one-year survival rate was similar for the ERCC1-negative patients (61%) and the ERCC1-positive patients (70%). CONCLUSION: In the current study, the tumor ERCC1 expression by IHC staining could not predict the clinical response or survival of AGC patients who were treated with cisplatin-based first-line chemotherapy. The ERCC1 protein expression does not appear to be a useful tool for the selection of tailored chemotherapy for these patients.

The Immunohistochemical Patterns of the beta-Catenin Expression in Pilomatricoma

BACKGROUND: Pilomatricoma is a benign follicular tumor that is composed of basaloid cells, transitional cells and shadow cells. beta-Catenin is a 92-kDa protein, and it plays important roles in cell-cell adhesion at the cell membrane and signal transduction in the nucleus. beta-Catenin has recently been shown to play an important role in the formation of hair follicle-related tumors, including pilomatricoma. However, the pattern and the intracellular localization of the beta-Catenin expression are still controversial. OBJECTIVE: We wanted to evaluate the pattern and the intracellular localization of the beta-Catenin expression in pilomatricoma by performing immunohistochemical staining. METHODS: Twenty-seven paraffin-embedded tissue samples that were diagnosed as pilomatricoma were immunohistochemically stained with beta-Catenin antibody. RESULTS: Basaloid cells were found 15 samples of the total 27 pilomatricomas. All (15/15) of the basaloid cells strongly expressed beta-Catenin, but the transitional cells and the shadow cells did not. In the basaloid cells, the nuclei and membranes showed prominent beta-Catenin immunoreactivities, but the cytoplasm showed weak beta-Catenin immunoreactivity. CONCLUSION: This study confirmed that the nucleus and membrane of all the basaloid cells in the pilomatricomas showed a strong beta-Catenin expression, but the transitional cells and shadow cells showed negative beta-Catenin immunoreactivity.

Esophageal Metastasis of Malignant Melanoma in a 66-year-old Female Patient

Malignant melanoma is a malignant neoplasm originating from the melanocytes, which are cells derived from the neural crest. Gastrointestinal metastasis that spreads through lymphatic or hematogenous routes is an important prog- nostic factor; however, esophageal metastasis has rarely been reported. A number of immunohistochemical stai- ning methods for the pathological confirmation of esophageal metastasis have been introduced recently. Metastatic malignant melanoma is treated with chemotherapy and immunotherapy, but the prognosis is very poor. We report the case of a 66-year-old female who presented with difficulty swallowing, epigastric discomfort, and multiple variable- sized esophageal nodules diagnosed as esophageal metastases from subcutaneous malignant melanoma.

Primary Granulocytic Sarcoma with Multiple Organ Involvement / 대한혈액학회지

We report here a case of primary granulocytic sarcoma that involved multiple organs simultaneously and simulated clinical features of lymphoma at initial presentation. A 55 year-old man was referred to our center for the treatment and evaluation of lymphoma, which was diagnosed for nasal and colonic polyps in a local hospital. In our center, brain MR imaging showed multiple soft tissue lesions in brain and the whole body PET demonstrated multiple hypermetabolic lesions. Immunohistochemical staining of biopsy specimen on nasal and colonic polyp showed negativity of all lymphoma markers and positivity of myeloid markers, and final pathologic diagnosis was granulocytic sarcoma. Cytogenetic analysis of malignant cells in CSF showed chromosomal abnormalities of t(16;16)(p13.1;q22). Bilateral bone marrow examination was done with no evidence of abnormal cell infiltration and with normal cytogenetics. Complete remission was induced with 5 cycles of anti-leukemic chemotherapy, intrathecal chemotherapy and whole brain irradiation. However, leukemia relapsed in blood and bone marrow three months after the completion of treatment and the patient died 11.5 months after initial diagnosis of primary granulocytic sarcoma. In conclusion, immune-histochemical staining is necessary to obtain accurate pathologic diagnosis of granulocytic sarcoma. Pathogenetic role of t(16;16) in granulocytic sarcoma should be evaluated and appropriate treatment of primary granulocytic sarcoma should be defined in the future studies.

Clinical Study of Intraductal Papillary Mucinous Tumor of the Pancreas

PURPOSE: Intraductal papillary mucinous tumor (IPMT) of the pancreas pathologically shows papillary proliferation and its tumor cells display a spectrum of changes ranging from adenoma to infiltrating carcinoma. Because of this variability, there have been many difficulties for making an accurate diagnosis and administering proper treatment. The aim of this study was to determine the treatment strategy and differential diagnosis of benign and malignant IPMT. METHODS: Between January 2000 and June 2007, 24 patients with IPMT of the pancreas underwent surgery. The relationships among the clinopathologic features and tumor locations and subtypes were retrospectively investigated. In addition, the type of surgical procedures and findings, the microscopic finding, the immunohistochemical staining and the clinopathological features were analyzed. RESULTS: There were 17 men and 7 women with a mean age of 65 (range: 45~81). Pathologically, 11 cases were benign, 9 were borderline and 4 were malignant. The tumor was located in the head of the pancreas in 17 patients. 16 cases received Whipple's procedure and pylorus preserving pancreaticoduodenectomy, and the others were received different kinds of operations. Regarding the subtypes of IPMT, 2 cases were the main duct type, 19 were the branched type and 3 were the combined type. There were no statistically significant differences in the clinical manifestations, radiologic findings and immnohistochemical staining between the patients with benign and malignant IPMT. Except two patients who were not followed up, all the patients had no recurrence and they survived. CONCLUSION: It is very difficult to exactly differentiate malignant IPMT from benign IPMT with using the current preoperative evaluations and immunohistochemical staining of the resected specimens. The patients who were operated on and followed in our hospital had no recurrence and they all survived. Therefore, if IPMT is suspected, we think the patients should be operated on and we should continue studying other specific antibodies for immunohistochemical staining.

The Prognostic Value of CA19-9 in Patients with Colorectal Cancer

PURPOSE: There has been much debate about the significance of the CA19-9 level for predicting the prognosis of colorectal cancer patients. This study aimed to evaluate the prognostic value of the preoperative serum CA19-9 level and the CA19-9 expression in the tumor tissues of colorectal cancer patients METHODS: One hundred patients with colorectal cancer and who had been treated by resection were studied. We assessed the correlations of the preoperative serum CA19-9 level and the status of the CA19-9 immunohistochemical staining with the clinicopathologic features, including the prognosis of the patients. RESULTS: The preoperative serum CA19-9 level had significant correlation with the status of CA19-9 immunohistochemical staining. The presence of distant metastasis was significantly correlated with an elevated level of serum CA19-9. The depth of tumor, the presence of lymph node metastasis, the TNM stage and tumor cell differentiation were significantly correlated with the status of the CA19-9 immunohistochemical staining. In addition, the gross morphology, depth of tumor, the presence of lymph node metastasis, the TNM stage, the status of the CA19-9 immunohistochemical staining and the serum CEA level were correlated with survival on univariate analysis. However, multivariate analysis did not validate the status of CA19-9 immunohistochemical staining as a significantly independent predictor of the prognosis. CONCLUSION: The CA19-9 expression was frequently observed in advanced stage tumor tissue, yet its expression in tumor tissue or the preoperative CA19-9 serum level did not show independent prognostic value for colorectal cancer patients.

Idiopathic Intestinal Pseudo-obstruction in Infants Surgically Treated: Findings to Help Diagnose Intestinal Neuronal Dysplasia and the Significance of Surgical Treatment

PURPOSE: Intestinal neuronal dysplasia (IND) causes intestinal pseudo-obstruction and shares clinical features with Hirschsprung's disease. Diagnosis of IND involves histopathological features of an intestinal biopsy, but diagnostic criteria are controversial and optimal treatment is unclear. We determined the pathological findings for diagnosing IND in infants and the significance of surgical treatment. METHODS: We retrospectively studied 4 patients who received bowel surgery for an intestinal obstruction without a definite obstructive cause that were subsequently diagnosed as IND by postoperative pathology. The clinical history and results of immunohistochemistry for ganglion and nerve fibers (NCAM, NSE, cathepsin D, synaptophysin) were compared between patients and control cases. RESULTS: All 4 patients were premature babies with symptoms of poor oral intake and abdominal distention. Surgical treatment was segmental resection of the small bowel in one case, segmental resection of the small bowel and double-barreled ileostomy in one case with NEC, and a temporary ileostomy for decompression and appendectomy for biopsy in 2 cases. The first 2 patients died of sepsis and DIC, respectively. The postoperative course of the other 2 patients was excellent for long-term follow up (30+/-6months). Patients with IND showed significantly more submucosal giant plexuses and ganglia in the submucosal plexus, a higher percentage of giant plexus in the 20 submucosal plexus, as well as increased incidence of heterotopic ganglia in the lamina propria, bud-like ganglia, anisomorphic ganglia, and immature ganglia. CONCLUSION: Proper surgical treatment of persistent intestinal pseudo-obstruction, including IND, can affect the prognosis and recovery of bowel function, with positive pathological findings helpful for diagnosing IND in infancy.

Prognostic Significance of Serum and Tissue Carcinoembryonic Antigen in Patients with Gastric Adenocarcinomas / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Carcinoembryonic antigen (CEA) is known to be elevated in nearly all solid malignancies. The prognostic role of CEA in gastric cancers however, is still controversial. We evaluated preoperative serum CEA levels and CEA expression from the resected tumor tissues to determine whether they have prognostic significance in gastric cancer patients. MATERIALS AND METHODS: Medical records of 810 patients who underwent surgery for gastric adenocarcinoma from June, 1998 to February, 2002 in Kyungpook National University Hospital were reviewed. Serum CEA level was evaluated by radioimmunoassay preoperatively, and the cut-off level for positivity was 7.0 ng/ml. Labeled streptavidin-biotin peroxidase method was used to determine CEA expression from the gastric cancer tissues. RESULTS: Serum and tissue CEA were positive in 9.3% and 91.1% of the patients, respectively. They had no correlation with each other. The positivity rate of serum CEA had positive correlation with invasion depth (p<0.001), lymph node metastasis (p<0.001), distant metastasis (p=0.006), and final stage (p<0.001). Well differentiated tumors showed higher serum CEA positivity (p=0.002). Patients with positive serum CEA had higher recurrence rate (p<0.001). Multivariate analysis showed significantly lower survival rate in patients with preoperative CEA levels over 7 ng/ml than those with lower levels (48.0% vs. 80.7%; p<0.001). The positivity rates of tissue CEA were higher in advanced cancers (p=0.033) and in more advanced stages (p=0.029). Tissue CEA positivity showed no correlation with recurrence or survival. CONCLUSIONS: Preoperative serum CEA level had correlation with disease progression and survival in gastric cancer patients, and proved to be an independent prognostic factor. Tissue CEA expression in gastric cancers had no prognostic information.

Neuroendocrine Tumor of the Colon

Neuroendocrine tumor (NET) of the colon and rectum has been reported to have a low incidence and aggressive progression; it is frequently misdiagnosed and its treatment is not well documented. Four NET cases were collected at our hospital during the previous year of a colon cancer survey. Endoscopic mucosal biopsy of the colon was done for each case and all the cases proved to be adenocarcinomas. Curative surgery was conducted after the preoperative diagnostic and staging evaluation was completed. The locations of the primary lesions of the patients were all different; cecum, ascending colon, splenic flexure colon and sigmoid colon. The disease was advanced in all cases and the first postoperative diagnosis was poorly differentiated adenocarcinoma. Immunohistochemical tests followed and all the cases were positive for chromogranin A staining. A retrospective study was then conducted.

A Case of Gastrointestinal Stromal Tumor in a Child / 대한소아소화기영양학회지

Gastrointestinal stromal tumors (GISTs) are the most common primary mesenchymal tumors of the digestive tract. They have been commonly observed in adults but have been rarely described in children. They arise typically from the intestinal wall and rarely in the mesentery, omentum, or retroperitoneum. GISTs originate from the interstitial cell of Cajal and are characterized by overexpression of the receptor tyrosine kinase c-kit. Up to 94% of these tumors express the CD117 on immunohistochemical stain. Surgery is the main modality of treatment for primary resectable GIST. Completely resectable GIST with low risk has excellent prognosis after primary surgical intervention, with over 90% of the 5-year survival. We report a case of 10-year-old girl presenting with an upper gastrointestinal bleeding caused by gastrointestinal stromal tumor.

A Case of Primary Small Cell Neuroendocrine Carcinoma of the Liver / 대한소화기학회지

Small cell neuroendocrine carcinoma is a type of undifferentiated, malignant neuroendocrine tumor. Most of neuroendocrine tumors exhibit well-differentiated features and are classified as carcinoid tumors. However, carcinomas of the liver with anaplastic characters, which are classified as small-cell carcinomas are extremely rare and only few cases have been reported in the literature. We report an unusual case of primary small cell neuroendocrine carcinoma of the liver in a 67-year-old man. The patient was found to have a palpable mass on right upper quadrant of abdomen on physical examination. The diagnosis was made by immunohistochemical stains of biopsied specimen from the liver. Other possible primary site was excluded by radiologic and endoscopic evaluations. The tumor was composed of small monotonous and hyperchromatic poorly differentiated cells with higher nuclear to cytoplasmic ratio, and were positive for neuroendocrine tissue markers such as synaptophysin, c-kit, and CD56.

Expression of Survivin and Its Correlation with Prognosis in Colorectal Cancer

PURPOSE: Survivin is involved in both the control of cell division and the inhibition of apoptosis. Specifically, its anti-apoptotic function is related to the ability to inhibit caspases directly or indirectly. This study examined the expression patterns of survivin in normal colorectal tissues and in colorectal cancer tissues to determine whether the expression of survivin is associated with either the colorectal cancer characteristics or the prognosis. METHODS: 4micrometer sections of the formalin-fixed paraffin-embedded samples of colorectal cancer tissues were the immunostained using antibodies for survivin. The immunostain was recorded as 0~3 depending on the stain intensity distribution in the cytoplasm and the nucleus. RESULTS: Survivin was localized in the nucleus and/or cytoplasm of tumor cells. We could differentiate between cytoplasmic and nuclear localization of survivin protein expression. Among the cancer expressions, 35.8% demonstrated nuclear staining, and 51.9% demonstrated cytoplasm staining. Statistical analysis revealed that cytoplasmic survivin expression was correlated with lymph-node metastasis, tumor stage, and patient survival. CONCLUSIONS: Survivin expression was correlated with clinicopathologic prognostic parameters and with the outcome. Thus, it can be both a useful diagnostic marker for colorectal carcinomas and an important source of prognostic information for patients with a colorectal carcinoma. Survivin will become a potential new target in anti-cancer therapy in near future.

Clinicopathological Characteristics of Kaposi's Sarcoma and Usefulness of Detection of Human Herpes Virus 8 / 대한피부과학회지

BACKGROUND: Kaposi's sarcoma (KS) is a multicentric proliferative vascular tumor which involves cutaneous and visceral tissues. Recent study has clearly identified human herpes virus 8 (HHV8) in all Kaposi's sarcoma patients, indicating that HHV8 is closely involved in the pathogenesis of Kaposi's sarcoma. OBJECTIVE: The purpose of this study was to document clinical and histopathological features of KS and to emphasize the necessity of detection of HHV8 in the differential diagnosis of KS from other vascular lesions. METHODS: The medical records and histopathological slides of patients with KS diagnosed at Ajou University Hospital from January 1995 to December 2004 were reviewed. We performed immunohistochemical stain and polymerase chain reaction (PCR)-based analysis to detect HHV8 in KS and other vascular lesions. RESULTS: Among 12 patients, classic KS was found in 9 patients, AIDS-associated KS in 1 patient, and iatrogenic immunosuppressive KS in 2 patients. Patients with KS presented with various clinical features, showing purple- colored macules to nodules or tumors. Although lower extremities are most frequently involved sites, involvement of other sites such as arm and neck was noticed. Mucosal and systemic involvement was detected in AIDS- associated case. Immunohistochemical stains for HHV8 were positive in all KS, but they were negative in other vascular lesions. PCRs for HHV8 were positive in 8 of 11 (72.7%) KS, but they were negative in other vascular lesions. Classic KS responded well to surgical and radiation therapies and showed indolent course. Immunosuppressive KS regressed partially after dose reduction of immunosuppressive drug therapy, but the lesions persisted. CONCLUSION: Immunohistochemical stain and/or PCR for HHV8 are useful means to differentiate KS from other vascular tumors.

Desmoplastic Small Round Cell Tumor: A Case Report / 영남의대학술지

Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant mesenchymal tumor found in the abdominal cavity. It mainly affects young male patients. We report a case of DSRCT that occurred in the abdominal cavity of a 50-year-old man. The tumor was characterized by small round tumor cells with irregular nests in the prominent desmoplastic stroma. The tumor cells showed immunoreactivity for epithelial membrane antigen, desmin, vimentin, and neuron specific enolase.

Histologic/Immunohistochemical Classification of Carcinoma of the Ampulla of Vater and Survival Rate

PURPOSE: The prognostic factors for carcinoma of the Ampulla of Vater have not been fully identified. This study was undertaken in order to determine whether or not immunohistochemical staining can be used identify the potential diagnostic and prognostic markers for carcinoma of the Ampulla of Vater. METHODS: We studied twenty four patients with carcinoma of the Ampulla of Vater who underwent curative resection from September 1995 to February 2003 at the Department of Surgery, Dong-A University Medical Center. All the specimens were reviewed by an expert pathologist, and immunohistochemical stains were used to differentiate the pathologic type. RESULTS: The overall 5-year survival rate was 58.3%, and survival was closely correlated with the pathologic differentiation. For the immunohistochemical stains, CK20 and MUC2 were significantly effective to differentiate the intestinal type and the pancreaticobiliary type of carcinoma for the Ampulla of Vater (P=0.0162 and P=0.0001, respectively). CONCLUSION: Further investigations with a larger number of cases would enable us to use immunohistochemical stains to improve the differentiation of carcinoma of the Ampulla of Vater.

A Family of Bethlem Myopathy

Bethlem myopathy (BM) is an early-onset benign autosomal dominant myopathy characterized by proximal muscle weakness and multiple contractures. It is caused by mutations in the three genes encoding collagen VI, which is a ubiquitous extracellular matrix protein forming a microfibrillar network in close association with the basement membrane. Here, we present a family with BM which is the first reported Korean case.