Successful Treatment with Definitive Concurrent Chemoradiotherapy Followed by Durvalumab Maintenance Therapy in a Patient with Tracheal Adenoid Cystic Carcinoma.

Adenoid cystic carcinoma (ACC) is a rare type of malignant tracheal tumor originating from the secretory glands. Complete surgical resection is the current standard of care for tracheal ACC. However, there have been few case reports of chemoradiotherapy for unresectable tracheal ACC. We herein report a 28-year-old man with unresectable tracheal ACC who received concurrent chemoradiotherapy (CCRT) followed by maintenance therapy with durvalumab. CCRT was completed with a good response and safety, and the patient is currently receiving durvalumab as maintenance therapy. Durvalumab after CCRT can be a treatment option for patients with unresectable tracheal ACC.

Alectinib-Induced Severe Hemolytic Anemia in a Patient with ALK-Positive Non-Small Cell Lung Cancer: A Case Report.

Alectinib is a selective anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitor as standard therapy for ALK-rearranged non-small cell lung cancer (NSCLC). Hemolytic anemia is considered as a rare but significant adverse event with alectinib. Here, we report a case of a 73-year-old female with lung adenocarcinoma, harbouring an ALK fusion gene, who received alectinib as second-line therapy and developed gradually progressive grade 4 (6.4 g/dL) drug-induced hemolytic anemia (DIHA) after complete response. We discontinued alectinib and performed a blood transfusion for the severe anemia. The anemia improved with no recurrence of lung adenocarcinoma over 10 months. Regular hematologic monitoring and the possibility of DIHA should be considered in case of progressive hemolytic anemia during alectinib treatment.

Repeated Resection for Recurrent Metastatic Solid Pseudopapillary Neoplasm of the Pancreas.

BACKGROUND Solid pseudopapillary neoplasm (SPN) accounts for 1.0% to 2.0% of all pancreatic neoplasms. SPN generally has good prognosis after surgery; however, 10% to 15% of patients have local recurrence or distant metastasis. There have been a few reports of successful surgical resection of isolated recurrent tumors after radical resection and sporadic reports of multiple metastasectomies. Herein, we present a case of recurrent SPN treated by repeated surgeries. CASE REPORT A 49-year-old woman was referred to our hospital with jaundice and right upper abdominal pain. Computed tomography (CT) scanning revealed a 73×43-mm heterogeneous mass in the pancreatic head. We performed a pancreatoduodenectomy and diagnosed SPN. The patient was discharged without any complications and was followed up by CT once every 6 to 12 months. Six years later, a 15×15-mm tumor was detected in Couinaud segment VI of the liver. A liver biopsy showed a pathological match to the pancreatic tumor. We performed a partial hepatectomy, and the pathology report confirmed metastatic SPN. At 8 and 10 years after the initial surgery, the patient underwent further partial hepatectomies for confirmed solitary liver metastases of SPN. The Ki-67 index increased for each metastasis identified (initial tumor, 1.88%; 6 years, 7.38%; 8 years, 5.53%; 10 years, 11.22%). No further masses were detected, and the patient survived more than 10 years following surgery. CONCLUSIONS Despite histological transformation to high-grade malignant disease, repeated aggressive surgical resection led to long-term survival in our patient with SPN.

A case of Williams syndrome with suspected coexisting ectopic aldosterone-producing tumor in the liver.

SUMMARY: A 31-year-old man with Williams syndrome (WS) was referred to our hospital because of a 9-year history of hypertension, hypokalemia, and high plasma aldosterone concentration to renin activity ratio. A diagnosis of primary aldosteronism (PA) was clinically confirmed but an abdominal CT scan showed no abnormal findings in his adrenal glands. However, a 13-mm hypervascular tumor in the posterosuperior segment of the right hepatic lobe was detected. Adrenal venous sampling (AVS) subsequently revealed the presence of an extended tributary of the right adrenal vein to the liver surrounding the tumor. Segmental AVS further demonstrated a high plasma aldosterone concentration (PAC) in the right superior tributary vein draining the tumor. Laparoscopic partial hepatectomy was performed. The resected tumor histologically separated from the liver was composed of clear cells, immunohistochemically positive for aldesterone synthase (CYP11B2), and subsequently diagnosed as aldosterone-producing adrenal adenoma. After surgery, his blood pressure, serum potassium level, plasma renin activity and PAC were normalized. To the best of our knowledge, this is the first report of WS associated with PA. WS harbors a high prevalence of hypertension and therefore PA should be considered when managing the patients with WS and hypertension. In this case, the CT findings alone could not differentiate the adrenal rest tumor. Our case, therefore, highlights the usefulness of segmental AVS to distinguish adrenal tumors from hepatic adrenal rest tumors. LEARNING POINTS: Williams syndrome (WS) is a rare genetic disorder, characterized by a constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. WS is a disease with a high frequency of hypertension but the renin-aldosterone system in WS cases has not been studied at all. If a patient with WS had hypertension and severe hypokalemia, low PRA and high ARR, the coexistence of primary aldosteronism (PA) should be considered. Adrenal rest tumors are thought to arise from aberrant adrenal tissues and are a rare cause of PA. Hepatic adrenal rest tumor (HART) should be considered in the differential diagnosis when detecting a mass in the right hepatic lobe. Segmental adrenal venous sampling could contribute to distinguish adrenal tumors from HART.

Midline extraperitoneal approach for bilateral widespread retroperitoneal abscess originating from anorectal infection.

INTRODUCTION: Anorectal abscess is one of the most common anorectal conditions encountered in practice. However, such abscesses may rarely extend upward and cause life-threatening medical conditions. PRESENTATION OF CASE: A 53-year-old woman presented with symptoms of anorectal abscess and evidence of severe inflammatory response and acute kidney injury. Computed tomography revealed a widespread abscess extending to the bilateral retroperitoneal spaces. Surgical drainage was performed via a totally extraperitoneal approach through a lower midline abdominal incision, and the patient had a rapid and uncomplicated recovery. DISCUSSION: Although retroperitoneal abscesses originating from the anorectal region are rare, they are life-threating events that require immediate treatment. Percutaneous abscess drainage has been recently evolved; however, surgical drainage is required sometimes that may be challenging, particularly in the case of widespread abscesses, as in our case. CONCLUSION: The midline extraperitoneal approach reported here might be an effective surgical option for patients with bilateral widespread retroperitoneal abscesses.

[Case of solitary necrotic nodule of the liver difficult to distinguish from malignant hepatoma].

In a 72 years old woman, abdominal computed tomography (CT) disclosed multicentric hypervascular tumor in S4 of the liver, and it was T1.T2 by the MRI, and a high signal was presented together. Stenosis was shown by ERCP at the same site. Cholangiocellular carcinoma was suspected before operation, but malignant change was not recognized by frozen section, so the operation was finished. Later, a diagnosis of solitary necrotic nodule of the liver was established by pathological diagnosis. Solitary necrotic nodule of the liver is a benign tumor.

Pseudomyxoma peritonei due to mucinous cystadenocarcinoma in situ of the urachus presenting as an inguinal hernia.

Pseudomyxoma peritonei is generally caused by appendiceal and ovarian tumors. Other primary sites have been rarely reported. We describe herein the second reported case of pseudomyxoma peritonei due to mucinous cystadenocarcinoma of the urachus. A 54-year-old man was admitted with a left inguinal hernia that had developed several months prior to his admission. During herniorrhaphy, we found a large amount of gelatinous mucinous material in the indirect-hernia sac and made a diagnosis of pseudomyxoma peritonei on cytological grounds. At re-operation, the origin of the pseudomyxoma peritonei proved to be a ruptured urachal cyst. The urachal cyst and the dome of the urinary bladder were excised. In addition, we removed as much of the gelatinous material as possible. On histological examination, a unilocular cyst was found to consist of noninvasive mucinous adenocarcinoma. We succeeded in removing the rest of the mucinous material by postoperative intraperitoneal lavage with dextran solution, and have observed no evidence of recurrence for 7 years since the operation.

Evaluation of malignancy using Ki-67 labeling index for gastric stromal tumor.

BACKGROUND: Assessment of malignant potential in gastrointestinal stromal tumors (GISTs) is still problematic. The maximum tumor diameter and the mitotic index are generally used as an index of malignancy of GISTs. The Ki-67 labeling index has recently been used as an index of cell growth, and the prognosis of GISTs was reported to be significantly poor when the value of this index was 10% or higher. METHODS: Clinicopathological and immunohistological factors were analyzed in 15 patients who underwent surgical resection of gastric stromal tumors at our department between April 1997 and July 2002. The patients were divided into "metastasis/recurrence" and "benign" groups. Also, the relationship of changes in the Ki-67 labeling index to the degree of malignancy in recurrent lesions was assessed in an 84-year-old woman who underwent five reoperations because of recurrences in the peritoneum. RESULTS: Significant differences were noted between the metastasis/recurrence and benign groups in relation to the mean maximum tumor diameter (186.7 +/- 80.8 mm vs 41.3 +/- 22.9 mm), mitotic index (88.3 +/- 5.0/50 high-power fields [HPF] vs 3.0 +/- 2.9/50 HPF), and the Ki-67 labeling index (11.4 +/- 2.5% vs 0.01 +/- 0.51%). In the patient who had metastasis to the liver 3.5 years after initial operation and underwent five reoperations before death, the intervals until detection of recurrence tended to be shortened gradually. The Ki-67 labeling index varied with each operation, and tended to be higher at the time of reoperations than at the initial operation. CONCLUSION: The maximum tumor diameter, mitotic index, and Ki-67 labeling index were useful as an index of malignancy for gastric stromal tumor. The efficacy of surgical resection alone may be insufficient in patients with disseminated metastasis to the peritoneum.

[A case treated successfully with low-dose CDDP and 5-FU for the treatment of liver and para-aortic lymph node metastases and second metastasis to anterior mediastinum lymph nodes from gastric cancer after gastrectomy].

A 64-year-old-male had recurrent paraaortic lymph node and liver metastases eight months after total gastrectomy and with distal pancreatectomy and splenectomy for advanced gastric cancer. Combined chemotherapy with 5-FU and a low-dose of CDDP was effective and the both lesions disappeared. Thirteen months later, a second recurrence of anterior mediastinum lymph node metastases occurred. After the same protocol, the lesions showed a partial response and lymph node dissection was performed. Histopathological examination showed that the resected lymph nodes had 99% necrosis and fibrotic change. Immunohistochemical examination of p53 of the primary gastric cancer showed negative staining. The patient has been followed for three years after the operation, and has no recurrent lesions.