Tuberculous peritonitis after conservative treatment for acute perforated appendicitis: a case report.

BACKGROUND: Interval appendectomy is widely recommended for patients with abscesses due to perforated appendicitis. A concomitant malignancy-related problem was reported after conservative treatment of acute appendicitis with abscess, but perforated appendicitis-associated tuberculous peritonitis was never reported. CASE PRESENTATION: A 67-year-old male patient with a laryngeal cancer history presented to our hospital with an acute appendicitis-associated ileal abscess. He was scheduled for an interval appendectomy after conservative treatment. Fortunately, the symptoms subsided, and the patient was discharged for a later scheduled appendectomy. However, after 3 months, he was readmitted to our hospital with fever and abdominal pain, and emergency surgery was performed, which was suspected to be peritonitis. Intraoperative results revealed numerous white nodules in the abdominal cavity. The condition was diagnosed as tuberculous peritonitis based on macroscopic results, later pathological findings, and positive T-SPOT.TB. The antituberculosis medications were effective, and the patient recovered and was discharged from the hospital 8 days thereafter. CONCLUSION: Patients, particularly those immunocompromised, may develop tuberculous peritonitis after conservative treatment for acute perforated appendicitis.

Myxoid type and non-myxoid type of intimal sarcoma in large vessels and heart: review of histological and genetic profiles of 20 cases.

Intimal sarcoma is one of the most common and well-known primary malignant neoplasms of the aorta and heart. The authors reviewed cases of intimal sarcoma from histological, immunohistochemical and genetic perspectives. Twenty cases of intimal sarcoma were retrieved. Immunohistochemistry and FISH of MDM2 and PDGFRA genes were performed. All 20 tumours were composed of spindle-shaped, stellate, oval or polygonal tumour cells with irregular hyperchromatic nuclei arranged in a haphazard pattern, accompanied by nuclear pleomorphism and frequent mitotic figures. Other histological findings were as follows: abnormal mitosis in 10 cases (50%), necrosis in 15 cases (75%), myxoid stroma in 12 cases (60%), cartilaginous formation in 1 case (5%), haemorrhage in 12 cases (60%) and fibrinous deposition in 14 cases (70%). The tumours were positive for MDM2 in 16 cases (80%), ERG in 4 cases (20%), alpha-smooth muscle actin in 6 cases (30%), desmin in 5 cases (25%) and AE1/AE3 in 4 cases (20%). Immunohistochemical positivity was focal in each case. Loss of H3K27me3 expression was noted in 2 cases (10%). MDM2 and PDGFRA gene amplifications were detected in 11 cases (55%) and 1 case (5%), respectively. Fisher's exact test revealed a significant correlation between MDM2 gene amplification and myxoid stroma (p = 0.0194). No parameters showed any association with the anatomical location of the tumours. It was suggested that myxoid histology of intimal sarcoma may be associated with MDM2 gene amplification and that intimal sarcoma may be divided into myxoid and non-myxoid types.

Carpal Tunnel Syndrome Due to Iatrogenic Amyloidosis After Domino Liver Transplantation From Hereditary Transthyretin Amyloidosis: A Case Report.

BACKGROUND: Carpal tunnel syndrome is the most common compression syndrome of the peripheral nerve. Transthyretin amyloidosis and dialysis-related ß2-microglobulin amyloidosis are known causes of carpal tunnel syndrome. CASE REPORT: A Japanese woman showed carpal tunnel syndrome 16 years after a domino liver transplantation (DLT) from the donor with hereditary transthyretin amyloidosis. DLT indication was congenital extrahepatic portosystemic shunt, and the patient had been put on maintenance hemodialysis because of chronic kidney disease 6 years before DLT. Moreover, the amyloid precursor protein of the patient was histologically confirmed not to be ß2-microglobulin, but transthyretin. CONCLUSIONS: The existence of amyloid was speculated when the patient who underwent DLT from hereditary transthyretin amyloidosis showed carpal tunnel syndrome. Additionally, elucidating the amyloid precursor protein when the patient has another cause of amyloidosis is necessary.

Conversion hepatectomy for advanced hepatocellular carcinoma after right portal vein transection and lenvatinib therapy.

BACKGROUND: Lenvatinib is a novel tyrosine kinase inhibitor that exhibits an antitumor effect on hepatocellular carcinoma (HCC). An established strategy that involves surgery and usage of lenvatinib for advanced HCC remains elusive. CASE PRESENTATION: A 58-year-old male patient with advanced HCC and untreated hepatitis B was referred to our hospital. The tumor at the right lobe was 10 cm in diameter with right portal vein thrombus. Because of the possible lung metastasis and concern about the remaining hepatic function after extended right hepatectomy, lenvatinib was initiated before surgery. After the confirmation of a sharp decrease of tumor markers during the 3-week lenvatinib therapy, only a right portal vein transection was done leaving the enlargement of the left lobe for improved post-hepatectomy liver function while lenvatinib therapy was continued. The laparotomy revealed that the tumor was invading the right diaphragm. After 7 weeks of lenvatinib administration after right portal vein transection, an extended right hepatectomy with resection of the tumor-invaded diaphragm was successfully done. The lung nodules that were suspected as metastases had disappeared. The patient has been doing well without any sign of recurrence for 1 year. CONCLUSION: The strategy involving the induction of lenvatinib to conversion hepatectomy including the portal vein transection was effective for advanced HCC.

A case of Trousseau's syndrome due to intrahepatic cholangiocarcinoma with an extremely high level of CA19-9.

BACKGROUND: Trousseau's syndrome is a cancer-associated thrombosis. Trousseau's syndrome with cholangiocarcinoma is a rare condition with poor prognosis. CASE PRESENTATION: A 59-year-old female was admitted to our hospital with abdominal pain, headache, and nausea. Abdominal enhanced computed tomography revealed liver tumor, splenic infarction, and bilateral renal infarction. Multiple acute cerebral infarctions were also detected by magnetic resonance imaging. Her preoperative serum levels of carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were > 120,000 U/mL and 589.6 ng/mL, respectively, which were extremely high. Histopathology after right hepatectomy revealed moderately differentiated adenocarcinoma consistent with intrahepatic cholangiocarcinoma. Her serum levels of CA19-9 were trending down to 9029.2 and 2659.8 U/mL at 1 and 3 weeks after surgery, respectively. However, at 7 weeks after surgery, her CA19-9 levels increased in the presence of positive imaging findings in the remnant liver, hilar lymph nodes, and peritoneal cavity. The initiation of combination chemotherapy including gemcitabine and cisplatin had a significant effect. The patient was doing well at 6 months after the surgery. CONCLUSION: This rare case of Trousseau's syndrome due to cholangiocarcinoma suggests that extremely high CA19-9 levels might be a pathogenic factor of this syndrome.

Histological analysis of infiltrating macrophages in the cerebral aneurysm walls.

A series of recent evidences suggested activated macrophages have broadly two distinct forms that possess opposite functions for the process of inflammation: classically activated macrophages (M1/kill macrophages) and alternatively activated macrophages (M2/repair macrophages) according to their functions and expression markers. To elucidate what roles those two phenotypes of macrophages play in the evolution of cerebral aneurysm, the presence of macrophages inside the aneurysm walls was assessed with an immunohistochemical approach. The portions of the aneurysm domes deflated after neck clipping were utilized for the further histological examinations, including immunostainings with five antibodies to identify macrophage subpopulations. In this study, contrary to the previous reports, the following various ratios of subtypes were observed in the aneurysm walls: M1 > M2 (2 cases), M1 < M2 (2 cases), M1 = M2 (3 cases). While M1-like macrophages have been typically regarded as a main driver of the degenerating process, these surprisingly richer presences of M2-like macrophages in the aneurysm walls suggests that an unrecognized biological process might be in play in aneurysm development.

[Collision Lung Cancer Consisting of Squamous Cell Carcinoma and Adenocarcinoma which Showed Identical Epidermal Growth Factor Receptor Mutation;Report of a Case].

We report a 79-year-old woman with collision cancer in the right middle lobe of the lung. She had a persistent abnormal shadow after treatment for pneumonia pointed out in right middle lung field on chest radiogram, and referred to our hospital. On examination, the chest computed tomography showed a pure-solid mass of 7.6 cm in diameter in right middle lobe of the lung which was thought to invade the superior pulmonary vein. She underwent a successful right pneumonecomty, and the postoperative course was uneventful. The tumor proved to be a collision cancer consisting of poor differentiated squamous cell carcinoma and invasive adenocarcinoma, lepidic predominanat by pathological examination. Epidermal growth factor receptor mutations (L858R) were found in both squamous cell carcinoma and adenocarcinoma of the tumor, possibly suggesting the same origin of both histological types.

Case of concurrent benign metastasizing leiomyoma in the lung and retroperitoneum, with a focus on its etiology.

We report a rare, simultaneous occurrence of benign metastasizing leiomyoma in the lung and retroperitoneum in a 49-year-old woman who had previously undergone myomectomy at 35 years of age and hysterectomy at 45 years of age for multiple recurrences of histologically benign uterine leiomyomas. At 49 years of age, computed tomography-guided biopsy indicated benign metastasizing leiomyomas in the lung. In addition, a retroperitoneal leiomyoma was found that was resected along with both the ovaries via laparotomy. No sign or symptom of recurrence was observed 5 years later. The coexistence of benign metastasizing leiomyoma in the lung and retroperitoneum following surgery for conventional leiomyomas has rarely been reported. Further, the nature and etiology of benign metastasizing leiomyoma are still not well understood. This case is therefore worth reporting, and exploring its etiology is important.

Stromal sarcoma of the breast with lung metastases showing a clinical complete response to doxorubicin plus ifosfamide treatment: report of a case.

A 29-year-old woman presented with a painful right breast tumor, measuring 15 cm in diameter, which had progressed rapidly over 3 months. Core needle biopsy of the tumor revealed a malignant mesenchymal tumor. A mastectomy was performed, and pathological examination of the tumor showed stromal sarcoma. Solitary pleural dissemination in the right lung was suspected, based on the computed tomography image taken before the operation. Two months after surgery, bilateral multiple lung nodules were demonstrated. Systemic chemotherapy with doxorubicin plus ifosfamide was performed, and 3 months later the lung metastases had disappeared. Moreover, there is still no sign of recurrence at 5 months after the initiation of the chemotherapy. Breast stromal sarcoma is very rare, accounting for less than 1% of mammary neoplasms, and the treatment strategy is not well established, especially regarding chemotherapy. This case demonstrates the effectiveness of chemotherapy with doxorubicin plus ifosfamide for lung metastases from breast stromal sarcoma.

[A case of mammary Paget's disease with early systemic recurrence after operation].

A 66-year-old woman visited our hospital because of redness and erosion of her right breast, and was diagnosed with mammary Paget's disease by histological examination of erosive skin. The patient had a simple mastectomy. The pathological diagnosis was mammary Paget's disease (8x7.5 cm) showing intraductal spread. The resected skin margin was negative. Partially dermal microinvasion and lymphatic involvement were found. Immunohistochemical analysis of this tumor showed ER negative, PgR negative, and HER2 positive (3+). Eighteen months after the operation, she presented with redness of the chest wall with edema of the right arm. Incisional biopsy on the chest mass revealed a local recurrence of mammary Paget's disease. In addition, she had carcinomatous lymphangiosis in the bilateral lung, nodal metastases in the ipsilateral axillary and mediastinal space, and contralateral breast metastases on CT. She was treated with twelve courses of weekly paclitaxel in combination with trastuzumab. A complete response was obtained eleven months after initiation of chemotherapy. A rare case of early systemic recurrence of mammary Paget's disease after curative operation is reported with a review of the literature.

Muir-Torre syndrome associated with endometrial carcinoma.

Muir-Torre syndrome (MTS) is an autosomaldominant skin condition of genetic origin, characterized by tumors of the sebaceous glands or keratoacanthomas that are associated with malignant visceral diseases. MTS associated with gynecologic malignancy has rarely been reported. Here we report a woman with no family history of colorectal cancer who developed endometrial carcinoma, stage 3a, at 49 years of age and at age 51 years, developed two skin tumors, a nasal squamous cell carcinoma and a sebaceous carcinoma of the right eyelid. The appearance pattern of these skin tumors suggested MTS. Although MTS associated with endometrial carcinoma is rare, patients with endometrial carcinoma should undergo evaluation for visceral malignancies (mainly colon cancer) and sebaceous skin lesions, regardless of whether or not there is a family history of colorectal cancer.

[A case of partial response in liver metastatic lesion from gastric endocrine cell carcinoma treated with TS-1].

Endocrine cell carcinoma (ECC) of the stomach is a rare pathological type with a poor outcome. Standard chemotherapy has not yet been established. We experienced a case of partial response in the liver metastasis from the gastric ECC treated with TS-1. The patient was a 68-year-old female. An upper GI series and gastroendoscopy revealed pyrolus stenosis and CT-scan showed liver metastasis. A non-curative total gastrectomy was performed to allow oral intake. Since the histopathological examination revealed that tumor cells were partially positive for chromogranin A on immunohistochemical study, we diagnosed tumor ECC of the stomach. The patient with liver metastasis was treated using TS-1, and CT-scan showed a remarkable decrease (PR) in the metastatic lesion. TS-1 administration can improve the clinical outcome for ECC of the stomach.

Induction of sarcomas by a single subcutaneous injection of 7,12-dimethylbenz[a]anthracene into neonatal male Sprague-Dawley rats: histopathological and immunohistochemical analyses.

Animal experiments have shown that carcinogenicity of chemicals is higher in fetal or neonatal periods than adult. We investigated sensitivities to a carcinogen in peri-neonatal rats with a model of sarcomas-induction by a subcutaneous injection of chemo-carcinogen that has rarely done in neonatal rats. Neonatal male SD rats were injected with 7,12-DMBA 10, 100, and 500 microg, which resulted in sarcomas-induction in 0, 62, and 94% of rats. Male SD rats were injected with DMBA 500 microg at 0, 3, 7, 14, and 21 days, which resulted in sarcomas-induction in 94, 70, 64, 50, and 44% of rats. Although the induced sarcomas were occasionally in mixed morphological feature as previous reports for sarcomas of rat, each was immunohistochemically in almost monotonous pattern, and classification was feasible. The incidence of rhabdomyosarcomas was higher in rats neonatally injected with a higher dose of DMBA than a lower dose, and in rats injected at peri-neonatal periods than later periods. In histological observations for the site of injection before overt sarcomas develop, clusters of atypical mesenchymal cells emerged as previous studies, but also those were immunohistochemically differentiated into rhabdomyocytes and other mesenchymal cells. We consider these findings may contribute a little to elucidation of process of sarcomas-induction in rats.

Increased interleukin-18 expression in bone marrow of a patient with systemic juvenile idiopathic arthritis and unrecognized macrophage-activation syndrome.

The aberrant induction of proinflammatory cytokines is considered to be crucial in the pathogenesis of systemic juvenile idiopathic arthritis and adult-onset Still's disease. Interleukin-18 (IL-18) in particular has been reported to be a candidate for the key cytokine in both diseases; however, the origin of IL-18 is unclear. To clarify the origin, we investigated specimens from various organs obtained during autopsy of a child with systemic JIA and macrophage activation syndrome, using immunohistochemical staining. Our results showed a high number of cells expressing IL-18 in the bone marrow but not in the other organs. This finding suggests that bone marrow is the origin of increased serum IL-18 and raises the possibility that other proinflammatory cytokines are also induced by IL-18 in bone marrow in this disease. Bone marrow may be an essential organ in the pathogenesis of systemic JIA.

Primary malignant melanoma of the male urethra.

We report a case of primary malignant melanoma of the male urethra in a patient whose penile shaft was successfully preserved, but who proceeded to acute renal failure (ARF) after interferon (IFN)-beta adjuvant immunotherapy. Primary malignant melanoma of the male urethra is rare and usually shows highly malignant potential. Therefore, urologists must often perform phallectomy, which impacts on the patient both sexually and mentally. A 64-year-old man presented at Saiseikai Sendai Hospital with asymptomatic gross hematuria and was diagnosed as distal urethral tumor. We predicted the highly malignant potential of this tumor from the urethroscopic finding and from urinary cytological examination. We did not select trans-urethral resection (TUR), but selected partial urethrectomy. This patient proceeded to ARF 1 month after natural IFN-beta treatment as an adjuvant immunotherapy. As IFN-beta rarely induces the delayed renal failure, urologists should be aware of renal dysfunction after IFN-beta therapy for the treatment of malignant melanoma.