Rapid Progression of Skin Sclerosis Following Surgery for Carpal Tunnel Syndrome: A Case of Diffuse Cutaneous Systemic Sclerosis.

Carpal tunnel syndrome (CTS) is a frequently encountered compressive neuropathy that is often treated surgically. Here, we present an unusual case of a 74-year-old female who developed a rapid emergence of skin sclerosis following CTS surgery. The condition was initially misdiagnosed as complex regional pain syndrome. However, since her skin condition progressed, she was referred to the rheumatology department. Subsequent evaluations confirmed the diagnosis of diffuse cutaneous systemic sclerosis, accompanied by interstitial lung disease. Treatment with mycophenolate mofetil did not notably alter the interstitial lung shadows but led to minor improvement in skin sclerosis. It is crucial to consider the possibility of rheumatic diseases in patients with unexpected postoperative symptoms.

Primary isolated bone marrow diffuse large B cell lymphoma with long-term complete remission.

Secondary bone marrow involvement of non-Hodgkin's lymphoma (NHL) is relatively common. However, primary isolated bone marrow involvement in NHL which was successfully treated and remains in complete remission (CR) for a long-term duration without any relapse is extremely rare. We herein report a patient of primary bone marrow diffuse large B cell lymphoma (PBML/DLBCL) who presented a prolonged high-grade fever and systemic purpura due to severe thrombocytopenia. The patient was successfully treated with systemic chemotherapy by R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and remains in CR at 8 years after the initial diagnosis. Review of the literature in PBML/DLBCL cases are also shown.

[A Case of Advanced Neuroendocrine Cell Carcinoma of the Stomach Treated with S-1 and Cisplatin Chemotherapy].

A 69-year-old man presented to our hospital because of epigastric pain. A type 2 lesion was seen in the lesser curvature of the antrum of the stomach. A moderately differentiated adenocarcinoma(human epidermal growth factor receptor 2-negative) was diagnosed by biopsy. Abdominal computed tomography showed a mass shadow 52mm in diameter in the pyloric region invading the surrounding organs, but no evidence of distant metastasis. Chemotherapy with S-1 and cisplatin(SP therapy)was initiated because of a diagnosis of locally advanced gastric cancer. After 2 courses of chemotherapy, the tumor shrinkage rate was 70%, confirming that treatment was effective. However, severe skin disorders developed, precluding the continuation of chemotherapy. Staging laparoscopy showed no evidence of peritoneal dissemination, but invasion into the superior mesenteric vein was noted. The tumor was resected by pancreaticoduodenectomy with partial resection of the venous wall. Pathological examination of the resected specimens provided a definite diagnosis of neuroendocrine cell carcinoma. As of 1 year and 7 months after surgery, there has been no observation of metastasis or recurrence. SP therapy was suggested to be a useful regimen for preoperative chemotherapy in patients with locally advanced neuroendocrine cell carcinoma.

[Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma].

A 75-year-old male presented with progressive lower abdominal discomfort. CT scan demonstrated hypertrophy of the intestinal wall, small bowel dilatation, and masses in the descending colon. Biopsy specimens of the jejunum and descending colon revealed widespread distribution of medium-sized atypical lymphocytes with an immunophenotype, positivity for CD3, CD8, CD56, TAI-1, granzyme B and TCRß, but negativity for CD4, CD5, CD20, CD30 and EBER-ISH. Type II enteropathy-associated T cell lymphoma (EATL; Lugano, stage IIE) was diagnosed. Subsequently, he received 6 cycles of chemotherapy with 2/3 dose CHOP and obtained complete remission. However, 18 months after the initial presentation, he presented with rapidly progressive mental deterioration. Gadolinium enhanced T1-weighted brain MR images showed multiple masses with mild heterogeneous enhancement. Brain biopsy revealed necrotic tumors composed of medium-sized atypical lymphocytes, positive for CD3, CD8, CD56, TIA-1, granzyme B and TCRß, but negative for CD4, CD20, and EBER-ISH. CT scan disclosed no evidence of systemic lymphoma relapse, indicating central nervous system relapse of EATL. Despite immediate high-dose chemotherapy with methotrexate, he died of disease progression. EATL is a rare disease with a very poor outcome, for which a validated standard treatment is still lacking. Further studies are needed to identify innovative therapies for treating EATL.

Optic nerve involvement of Waldenström's macroglobulinemia: with autopsy findings.

Waldenström's macroglobulinemia (WM) is an indolent chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma (LPL), characterized by a proliferation of plasmacytoid lymphocytes and the production of monoclonal IgM. Although, peripheral neurologic complications commonly occurs due to hyperviscosity in WM, central nervous system (CNS) involvement is very rare. Herein, we present the case of a 67-year-old man who initially presented with progressive visual loss and was diagnosed as WM/LPL with a very aggressive clinical course. He underwent chemotherapy with high dose methotrexate (MTX) plus cytarabine (Ara-C). However, he died and findings of a subsequent autopsy revealed the presence of lymphoplasmacytoid cells in the optic nerve.

Primary B-cell lymphoma of the cauda equina, successfully treated with high-dose methotrexate plus high-dose cytarabine: a case report with MRI findings.

Primary malignant lymphoma of the cauda equina is an extremely rare disease. Previously, there have been only 12 reported cases of malignant lymphoma of the cauda equina, and most cases relapsed early in the clinical course. So, the optimal treatment strategy for this condition has not been established yet and the prognosis is thought to be poor. We experienced a case of B-cell malignant lymphoma of the cauda equina, with rapid progression of the muscle weakness of the bilateral lower extremities, successfully treated with high-dose methotrexate plus high-dose cytarabine (Ara-C) chemotherapy, followed by radiotherapy and in complete remission without any recurrence signs, 1.5 years after the initial diagnosis. Intrathecal chemotherapy with MTX, Ara-C, and predonisolone was simultaneously performed. We should carefully continue to monitor the clinical course of our case, with the examinations of magnetic resonance imaging and cerebrospinal fluid in order not to overlook any subtle neurological changes or other clinical symptoms.

Is histopathological evidence really essential for making a surgical decision about mucinous carcinoma arising in a perianal fistula? Report of a case.

We report an unusual case of mucinous adenocarcinoma of the anus associated with a chronic anal fistula, treated successfully by abdominoperineal resection (APR). Although multiple biopsies failed to reveal any histological evidence of malignancy, cancer was diagnosed from the mucin obtained for cytology. Subsequent histological examination of the resected specimen revealed clusters of cancer cells floating in a mucous lake, suggesting that it would have been difficult to acquire the cells in a biopsy sample. Conversely, the presence of mucin lakes and globules in specimens drained from the region of perianal sepsis may have been histologically informative for diagnosis. Thus, although biopsy of the lesion is undoubtedly essential for diagnosis, it often fails to provide enough information to make a definite diagnosis of mucinous carcinoma. This case illustrates that clinicians should base their decision on whether to perform surgery on clinical manifestations, imaging findings, and cytology of mucin obtained by drainage when it is difficult to obtain malignant cells by biopsy.

Pancreatobiliary fistula associated with an intraductal papillary-mucinous pancreatic neoplasm manifesting as obstructive jaundice: report of a case.

We report a pancreatobiliary fistula caused by an intraductal papillary-mucinous pancreatic neoplasm (IPMN), manifesting as obstructive jaundice. Computed tomography showed dilatation of the bile duct and main pancreatic duct, with multiple cystic masses in the head of the pancreas. Endoscopic retrograde pancreatocholangiography showed a patulous papilla with mucin secretion. Contrast enhancement outlined amorphous material obstructing the lower part of the common hepatic duct. Pancreatogram and magnetic resonance cholangiopancreatography showed diffuse dilatation of the main pancreatic duct and side branches without communication with the adjacent organs or duct. We performed pancreaticoduodenectomy for IPMN of the pancreatic head and a tumor-like lesion in the lower common bile duct (CBD). Macroscopically, impacted thick mucus protruded into the CBD from the pancreas via a pancreatobiliary fistula. Histologic examination revealed a pancreatobiliary fistula caused by intraductal papillary-mucinous carcinoma of the pancreas with mucin hypersecretion, an adenoma without interstitial infiltration, and isolated implantation of an IPMN in the bile duct mucosa around the fistula.

Necrotizing fasciitis secondary to carcinoma of the gallbladder with perforation.

We present an unusual case of necrotizing fasciitis in the upper abdominal wall caused by penetrating perforation of the gallbladder. It was manifested as an elastic and reddish abdominal swelling with severe tenderness, but no peritoneal irritation. Computed tomography (CT) demonstrated water density with a slightly elevated CT value and air bubbles in the subcutaneous space. The preoperative diagnosis was subcutaneous abscess with fasciitis. At surgery, necrotizing fasciitis and subcutaneous abscess secondary to penetrating perforation of the gallbladder were revealed. Cholecystectomy and peritoneal irrigation were performed. Although no tumor was evident during surgery, a tumor located close to the perforation site was found just after the operation. Pathological examination revealed gallbladder carcinoma without stones. There have been very few previous reports of necrotizing fasciitis following gallbladder perforation. The presentation, diagnosis, and management of fasciitis, as well as carcinoma of the gallbladder with perforation, are discussed.

Advanced gastric endocrine cell carcinoma with distant lymph node metastasis: a case report and clinicopathological characteristics of the disease.

Gastric pure endocrine cell carcinoma (ECC) is extremely rare. ECC occasionally shows multidirectional differentiation; that is, adenocarcinomatous and/or squamous proliferation. Because gastric ECC has aggressive biological behavior and shows frequent metastasis to liver and lymph nodes even in the early stage, the prognosis of patients having this disease is extremely poor. We treated a 75-year-old woman with advanced gastric pure ECC with total gastrectomy and lymph node dissection, and reviewed all the previously reported cases of this disease. We compared the clinicopathological findings of ECC with those of gastric carcinoma (GC) and found that ECC had significantly more frequent invasion to lymphatic and vascular lumens ( P < 0.01) and more frequent metastasis to lymph nodes ( P < 0.01) and liver ( P < 0.05) compared to GC. Gastric ECC smaller than 5 cm in the greatest dimension showed a higher percentage of advanced lesions (>T2) than GC ( P < 0.05), which could result in the difficulty of finding early ECC. The findings of the analyses we made in this report may account for the poor prognosis of this disease.

Expression and prognostic roles of beta-catenin in hepatocellular carcinoma: correlation with tumor progression and postoperative survival.

PURPOSE: Although hepatocellular carcinoma (HCC) is the most common primary malignant tumor of the human liver, the molecular changes and mechanisms that regulate its development and progression remain unclear. In the present study, we investigated the correlation between beta-catenin expression and clinical outcome in 51 patients with relatively small (maximal diameter < 30 mm), solitary HCCs. EXPERIMENTAL DESIGN: The tumors were classified according to histological tumor differentiation (grade I, 11 tumors; grade II, 28 tumors; grade III, 12 tumors). Using immunohistochemical methods to detect nuclear accumulation of beta-catenin, we investigated the correlation between beta-catenin expression and clinical outcome and compared the correlation with cyclin D1, Ki-67, and E-cadherin. RESULTS: Focal or generalized nuclear beta-catenin expression was observed in 36.4% (4 of 11) of the grade I tumors, 39.3% (11 of 28) of the grade II tumors, and 25% (3 of 12) of the grade III tumors. Nuclear beta-catenin-positive grade III tumors were associated with significantly poorer survival (P = 0.004), whereas none of the patients with nuclear beta-catenin-negative grade I tumors died. With regard to proliferative activity, positive nuclear beta-catenin staining correlated significantly with an increased Ki-67 labeling index in grade I (P < 0.0001) and grade III (P = 0.0045) tumors and with reduced epithelial cadherin expression in the cell membrane (P < 0.001). In contrast, no association with the expression of cyclin D1, one of the target factors of beta-catenin, was detected. CONCLUSIONS: Our present data suggest that beta-catenin plays important roles in promoting tumor progression by stimulating tumor cell proliferation and reducing the activity of cell adhesion systems and is associated with a poor prognosis, especially in patients with poorly differentiated HCCs.

Overexpression of MDM2 oncoprotein correlates with possession of estrogen receptor alpha and lack of MDM2 mRNA splice variants in human breast cancer.

To evaluate the significance of murine double minute 2 (MDM2) oncoprotein in human breast cancer as a nuclear-cytoplasmic shuttling protein, an estrogen receptor (ER) alpha regulator, and a prognostic marker and to study how MDM2 is overexpressed, we investigated its status in tissue samples and examined the correlation between overexpression and MDM2 gene abnormalities, status, and clinicopathological parameters. We detected MDM2 oncoprotein in both nucleus and cytoplasm by frozen-section immunohistochemistry. There was a significant correlation between MDM2 overexpression and low-grade nuclear atypia, absence of lymph node involvement, and increased levels of ER alpha protein. Our molecular assays found no point mutations in Ser17, but there was a correlation between MDM2 overexpression and the lack of splice variant mRNAs. These results suggest that the distribution of MDM2 reflects its nuclear-cytoplasmic shuttling ability; that interaction between p53 and MDM2 for tumor progression is not enhanced by point mutations at codon 17; and that the expression of MDM2 splice variants is a reason for the lack of its overexpression. MDM2 overexpression correlates with favorable prognostic parameters. A decreased level of MDM2 will lead to a deviation from the ER alpha signaling pathway.