Recurrence of inflammatory myofibroblastic tumor in bladder secondary to prostate treated with laparoscopic radical cystectomy.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare borderline tumor. The nomenclature of this disease is confused in the literature. CASE REPORT: In this report, the case of a 62-year-old man with IMT recurrence of bladder secondary to prostate is presented. The possible etiology of IMT is discussed, along with its clinical manifestation and pathological features. The patient received a laparoscopic bladder radical resection. The pathology finding demonstrated the diagnosis of IMT and no regional lymph node involvement. CONCLUSIONS: IMT is a borderline tumor and unlikely to metastasize to regional lymph nodes. The patient has been observed for 2 years without recurrence.

[Meduloblastoma: infrequent onset in local recurrence]. / Meduloblastoma: una presentación infrecuente en la recurrencia local.

INTRODUCTION. Meduloblastoma is high potential to recur and spread tumour. CASE REPORT. One case posterior fosse meduloblas toma in six years old boy who received 3 surgeries, chemotherapy and radiotherapy, with local recurrence and extracranial extension into neck laterocervical musculature. CONCLUSION. Although meduloblastoma extraneural metastases are not isolated phenomenon, meduloblastoma extracraneal extension is uncommon phenomenon in local recurrence.

[An example of metastasis to the iliopsoas muscle from sigmoid colon cancer].

Even though skeletal muscle, making up about 40% of body weight, is the largest organ in the human body, metastasis from malignant lesions is rare. Among reports of metastasis, those involving the iliopsoas muscle are numerous but few reports involve resection. Reported here is one example we experienced where metastasis developed in the iliopsoas muscle following colectomy, resection was then made possible by radiotherapy and chemotherapy. The case involved a 71-year- old male in which a Hartmann procedure was performed for sigmoid colon cancer. The pathology was Type 3 (8 × 7 cm, adenocarcinoma( mod), ss or more, ly1, v1, n0, ow(-), aw or ew(+), stage II). Upon additional sampling, thermal degredation of neoplastic cells was shown and outpatient oral UFT was performed. Five years following surgery, lymphoadenopathy was noted in the area of the left iliac artery upon US and CT. PET showed a probable metastasis to the left iliopsoas muscle. Early esophageal and stomach cancer were diagnosed by GFT. The esophageal cancer was located 30-32 cm from the incisors, unstained, Borrmann type 1, penetrating deeper than the muscularis propria. Biopsy revealed a diagnosis of tubular adenocarcinoma. ESD was performed for the esophageal cancer and one month later, a total gastrectomy D1+ß was performed. During surgery, the iliopsoas muscle tumor was determined to be large and impossible to resect. Radiotherapy of 10MV X-ray, 8 fields, 65-70 Gy/26 times for 6 . 5 weeks was performed for residual tumor but had no effect on tumor size. Fifteen courses of FOLFOX+bevacizumab were then performed. The tumor was markedly reduced in size, unidentifiable upon CT but showed slight uptake on PET and resection of the suspected residual tumor was performed. Histologically, atypical cells were shown in scarred muscle and connective tissue, however, degradation by chemotherapy was high. Residual tumor at resection margins was found, findings consistent with metastasis form sigmoid colon cancer. Taking into account the age and condition of the patient following surgery, chemotherapy was changed to S-1. Currently, 5 months after resection, there has been no recurrence.

[Exceptional bilocular inflammatory myofibroblastic pseudotumour (IMT) characterized by recurrent thoracic tumour growth and a rare (adult) second manifestation within the mesenteric tissue of the small intestine]. / Ungewöhnlicher bilokulärer inflammatorischer myofibroblastärer Pseudotumor (IMT), charakterisiert durch thorakales Rezidiv und seltene, da adulte, Zweitmanifestation im Dünndarmmesenterium.

BACKGROUND: Despite its rare occurrence, inflammatory myofibroblastic pseudotumour (IMT) is relevant in the differential diagnosis of intestinal lesions. By the mean of an extraordinary case report, tumour site and specific characteristics, finding of the correct diagnosis, therapeutic management, and outcome of extrapulmonary IMT is decribed based also on relevant references from the literature. CASE REPORT: A 39-year old man experienced a multifocal thoracic recurrence and abdominal metastasis of IMT 12 years after successful primary resection of pulmonary IMT. The intra-abdominal lesion localised in the jejunal mesenteric tissue was removed surgically (resection status, R 0) by segmental resection of the mid-jejunum (length: 80 cm) followed by jejunojejunostomy. Histology evaluation confirmed IMT. Thoracic surgeons advised against a surgical approach to the pulmonary and thoracic lesions because of their number and proximity to the superior vena cava as well as mediastinal infiltration. Despite receiving repeated advice from his physicians, the patient has not agreed to combined immunosuppressive treatment with cyclophosphamide and steroids, because of his desire for children. He underwent 5 months of systemic steroid treatment, starting in the third postoperative month, which he then chose to stop because of Cushing symptomatology. He agreed to a computed tomography (CT) scan follow-up 12 months after surgery, which revealed slight local progression of the remaining pulmonary lesion. Administration of a second steroid medication was initiated at a lower dose. No further CT scans were obtained. At present, he is consulting with an alternative medicine practitioner. CONCLUSION: This report documents a rarely described case of IMT at a jejunal mesenteric tumour site, interpreted as an uncommon late and extraordinary, metastatic, multifocal recurrence found 12 years (!) after surgical resection of the primary pulmonary tumour.

High expression of MMP14 is associated with progression and poor short-term prognosis in muscle-invasive bladder cancer.

OBJECTIVE: To evaluate the short-term prognostic value of matrix metalloproteinase 14 (MMP14) in muscle-invasive bladder cancer (MIBC). PATIENTS AND METHODS: Expression of MMP14 and clinical information from The Cancer Genome Atlas (TCGA) were mined in MIBC patients to analyse expression differences and conduct survival analyses. The mRNA and protein expression levels of MMP14 in other tumours were analysed using Gene Expression Profiling Interactive Analysis (GEPIA) and The Human Protein Atlas. The expression level of MMP14 in bladder cancer (BC) cell lines and clinical samples and its clinical significance were indicated using quantitative Real Time-Polymerase Chain Reaction (qRT-PCR), Western blotting, and immunohistochemistry. The biological functions of MMP14 were investigated by examining cell migration using in vitro wound-healing assays and cell invasion using transwell invasion assays. Survival analyses were conducted with the collected clinical follow-up data. RESULTS: Our study revealed that MMP14 is highly expressed in MIBC based, on both TCGA derived data and our clinical tissues (p<0.05). MMP14 is also highly expressed in head and neck cancer, renal cancer, pancreatic cancer and other cancers, as analysed using GEPIA and The Human Protein Atlas (p<0.05). Survival analyses of the TCGA data and our clinical follow-up data revealed high expression of MMP14 indicates a poor short-term prognosis in MIBC (p<0.05). Furthermore, downregulation of MMP14 suppressed BC cell invasion and migration abilities in vitro. MMP14 expression was closely correlated with tumour metastasis (p<0.05). T stage [hazard ratio (HR)=1.412, 95% confidence interval (CI)=1.121-1.779, p=0.003] and metastasis (HR=2.256, 95% CI=1.242-4.100, p=0.008) were unfavourable prognostic factors in BC patients. CONCLUSIONS: In MIBC, MMP14 expression is upregulated and closely associated with disease progression and poor short-term prognosis.

Tissue factor expression correlates with disease-specific survival in patients with node-negative muscle-invasive bladder cancer.

Tissue factor (TF), a transmembrane glycoprotein responsible for initiating the extrinsic pathway of blood coagulation plays a key role in cancer growth, metastasis and angiogenesis. Various studies have demonstrated the prognostic potential of TF expression in several cancers. However, its role in bladder cancer is unclear. This study evaluated the prognostic potential of TF expression in muscle-invasive bladder tumors from patients treated with radical cystectomies. Immunohistochemical staining using a monoclonal antibody (mAb) anti-TF was carried out on sections of tissue microarray blocks containing cores of muscle-invasive bladder tumors (4 cores/tumor) from 218 patients. The intensity of the staining was evaluated on a scale from 0 to 3 by two independent observers who were both unaware of the clinicopathological characteristics of the samples. TF was expressed in 77.6% of tumors, independently from baseline characteristics (age, gender, stage and grade) as assessed using the chi(2) and Student t tests. During follow-up (median: 2.6 years), 45.4% of the patients died from the progression of their cancer. Kaplan-Meier survival showed that among the 103 patients with node-negative (N0) transitional cell carcinoma (TCC), those with TF-positive tumors had shorter bladder cancer-specific survival (p = 0.0276). Moreover, multivariate Cox regression analysis showed they had a 3.15-fold greater risk of dying from bladder cancer (95% CI: 1.1-9.0; p = 0.032). In conclusion, TF expression was an independent predictor of disease-specific survival in N0 muscle-invasive TCCs treated by radical cystectomy and therefore, might help identify patients at higher risk of disease progression. These patients could potentially benefit from adjuvant chemotherapy.

Hybrid operating room Dyna-computed tomography combined image-guided electromagnetic navigation bronchoscopy dye marking and hookwire localization video-assisted thoracic surgery metastasectomy.

Hybrid operating room Dyna-computed tomography imaging is increasingly recognized as an important adjunct for management of small pulmonary lesions, either for biopsy or for localization to guide surgical resection. We describe for the first time the combined hybrid operating room Dyna-computed tomography image-guided electromagnetic navigation bronchoscopy dye marking and hookwire localization approach to localize multiple sub-centimetre nodules for video-assisted thoracic surgery metastasectomy. Furthermore, such complex set of procedures can be safely and effectively performed within one operating environment, maximizing work efficiency and minimizing the potential for complications.

Myofibrosarcoma: a clinicopathologic study.

The concept of soft tissue sarcomas composed predominantly of myofibroblasts has been controversial. We examined a series of such lesions of low- and intermediate-grade malignancy to further define their clinical and pathologic features. Histologic appearances of four cases diagnosed as myofibrosarcoma by electron microscopy were reviewed. Eleven additional cases with similar morphology were then identified from 249 tumors originally indexed as fibrosarcoma. Electron microscopy was performed on five of these, and immunohistochemistry was carried out on all cases for which material was available. There were 11 men and 4 women aged 33 to 73 years (median, 54 yrs; mean, 53 yrs). Lesions mainly involved the head and neck, extremities, and trunk and ranged in size from 1.5 to 12 cm. The tumors were composed of bland or pleomorphic stellate to spindled cells with eosinophilic cytoplasm and tapered nuclei in a collagenous stroma; 10 were grade 1 and five were grade 2. All sarcomas displayed fascicular or storiform patterns, and some of the grade 1 lesions superficially mimicked nodular fasciitis. Electron microscopy of nine cases showed myofibroblastic differentiation, and immunohistochemistry showed smooth muscle actin in 13 of 15 cases, muscle-specific actin in 7 of 9, desmin in 6 of 14, and cytokeratin in 0 of 11. Four of nine grade 1 and three of four grade 2 tumors recurred (one twice), and one grade 2 tumor metastasized to the lungs. Myofibrosarcomas are indolent low-grade or occasionally aggressive intermediate-grade sarcomas which can be recognized by light microscopy. Their clinical importance lies in the resemblance, particularly of low-grade examples, to reactive or pseudosarcomatous conditions.

Intraskeletal myofiber metastasis of breast carcinoma.

The authors report a case of chest skeletal muscle involvement by recurrent adenocarcinoma of the breast, in which individual myofibers were invaded by breast carcinoma cells. This unusual type of skeletal muscle involvement is especially interesting because it is known that the sarcolemma of skeletal myofibers is very resistant to enzymatic, mechanical, and chemical breakage.

The origin of the pseudoglandular spaces in metastatic smooth muscle neoplasm of uterine origin. Report of a case with ultrastructure and review of previous cases studied by electron microscopy.

The entity known as "leiomyomatous hamartoma," a term that has been used in reference to metastatic smooth muscle neoplasms of uterine origin (MSMNUO), is uncommon. Several articles have dealt with clinical and light microscopic aspects of this lesion. Four reports on the ultrastructure of this type of neoplasm have been published, but they have been primarily concerned with its smooth muscle component. Much controversy exists as to whether the glandular elements are part of the neoplastic process or preexisting pulmonary elements. This ultrastructural study confirms that the gland-like spaces represent entrapped alveoli and terminal respiratory bronchioles.

Locally metastasizing vascular tumor. Spindle cell, epithelioid, or unclassified hemangioendothelioma?

The authors report the case of a 2-year-old boy with a recurrent and locally metastasizing, spindle cell, vascular tumor with histiocytoid cells involving the skin, subcutaneous tissue, and muscle of the right forearm, the right distal radius and ulna, and multiple lymph nodes of the right axilla. Diagnoses of hemangioma, hemangiopericytoma, angiomatosis, spindle cell hemangioendothelioma, and malignant hemangioendothelioma were made on successive excision specimens. The soft tissue of the right arm became diffusely enlarged, and a severe syndrome developed that was similar to that described by Kasabach and Merritt. The limb was amputated above the elbow, the axillary lymph nodes were cleared, and a total dose of 6,000 centigrays axillary radiation was given. After operation, the Kasabach-Merritt syndrome resolved. Despite the lymph node metastases and multiple tissues involved, the patient has remained well 6 years after surgery. Although the tumor exhibited some of the histologic features of a spindle cell hemangioendothelioma, the low-grade aggressive behavior resembled that of an epithelioid hemangioendothelioma. The reported case cannot be classified into any of the recognized categories or subdivisions of vascular tumors.

Metastatic pattern of CC531 colon carcinoma cells in the abdominal cavity: an experimental model of peritoneal carcinomatosis in rats.

BACKGROUND: Peritoneal spread of tumour cells is a major source of morbidity and mortality in patients with colorectal cancer. In order to develop strategies to prevent intraperitoneal dissemination and to treat peritoneal carcinomatosis, the spread of tumour cells in the peritoneal cavity was studied. METHODS: Two million CC531 colon carcinoma cells were administered intraperitoneally in five groups of eight rats. The rats were killed after 1, 2, 4 and 8 hours and 3, 7, 14 and 21 days. After inspection of the abdominal cavity, samples of blood and ascites were taken. Liver, spleen, omentum, mesentery, diaphragm, parathymic lymph nodes and lungs were removed for histology and immunohistochemistry. RESULTS: No abnormalities were seen in the abdominal cavity until day 3. Subsequently the peritoneum and omentum became thickened and after 21 days all rats had haemorrhagic ascites and peritoneal carcinomatosis. The abdominal fluid contained tumour cells at all stages. The number of tumour cells decreased in the first 8 hours, and increased thereafter. At microscopy the peritoneum was completely covered by tumour cells after 3 days. Tumour cells concentrated in the milky spots (MS) of the omentum within 4 hours. The size of the MS increased as a result of an increase in number of tumour cells and macrophages. After 7--21 days the MS were completely replaced by tumour cells and new MS were formed. In the diaphragm tumour cells invaded the lymphatic lacunae after 8 h, and obliterated these after 3--7 days. Also invasion of the muscle fibres was seen after 3 days. Microscopically no tumour cells were found in blood, liver, spleen, parathymic nodes and lung. CONCLUSION: After intraperitoneal administration of CC531 colon carcinoma cells, tumour cells spread throughout the abdominal cavity, and concentrate in the milky spots of the greater omentum, the paracolic gutters, the subhepatic and subphrenic spaces and in the lymphatic lacunae of the diaphragm.

Metastatic low-grade inflammatory myofibroblastic tumor (IMT) in the central nervous system of a 29-year-old male patient.

A case of myofibrosarcoma (IMT) of the brain and lung as well as the spinal cord is described. A 29-year-old male patient presented with fever (40 degrees C), malaise, vomitus, meningism and leukocytosis. Computer tomography identified a bleeding in the left frontal lobe. A bleeding angioma was suspected and an operation was performed. The histological examination could not reveal an exact diagnosis. Eight months after complete recovery from the first bleeding, the patient had a second intracranial temporo-occipital bleeding on the right side which has been removed operatively. A new lesion was seen in the left parietal white matter of the brain. A growing cavernoma was suspected and resection of the lesion was planned. Pre-operatively the patient suffered from hemoptysis and fever. The X-ray of the chest showed a pulmonary lesion in the left lower lobe. In the CT of the chest a large tumor in the left lower lobe of the lung and additionally a cystic structure in the mediastinum was seen. The histological examination of this tumor identified an inflammatory myofibroblastic tumor (IMT). The left parietal lesion has been resected after the thoracic operation. The brain lesions were estimated to be metastases of the IMT of the lung. In the further clinical history the patient developed a large spinal cord metastasis of the thoracic spine. The metastatic development of the tumor reported in this case is unusual. The current therapy of these tumors consists of complete tumor resection and further clinical controls. However, due to the localization and the extension of some lesions in the present case, the complete resection has not been possible. There is no proven role of chemotherapy and radiation therapy. The patient died due to the pulmonary deterioration.

Malignant granular cell tumor.

A case of a granular cell tumor with a malignant behavior is described. Several repeated biopsy specimens, a resection specimen, and the metastatic lesions were compared with one another. Consecutive specimens showed increasing atypia. Electron microscopy of the metastatic lesions confirmed the granular cell nature of the tumor. The historic aspects of histogenesis are briefly discussed. The findings agree with a presently accepted origin from a primitive mesenchymal cell.

Orthotopic liver transplantation for inflammatory myofibroblastic tumor of the liver hilum.

Inflammatory myofibroblastic tumor is a rare gastrointestinal neoplasm. The authors report the case of a 5-year-old girl who initially presented with gastric outlet obstruction secondary to an inflammatory myofibroblastic tumor along the lesser gastric curvature. A subtotal gastrectomy and wedge resection of a left hepatic lobe nodule were performed. Obstructive jaundice developed one month postoperatively. Computerized tomography, percutaneous transhepatic cholangiography, and selective celiac and mesenteric arteriography showed a mass that involved the left hepatic lobe, with concomitant high-grade biliary obstruction and portal venous encasement. Percutaneous biliary drainage was performed. During laparotomy, the tumor was found to be unresectable, and the patient was referred to the Liver Transplant Service at The Children's Hospital of Philadelphia. A total hepatectomy with temporary portocaval shunt was performed, leaving the inferior vena cava in situ. At the back table, an ex vivo left trisegmentectomy was performed, followed by reimplantation of the posterior segment of the right hepatic lobe. The reimplanted liver segment functioned poorly, and completion hepatectomy with portocaval shunt was performed 24 hours postoperatively, because of severe coagulopathy, intraabdominal bleeding, and hemodynamic instability. The patient's condition stabilized, and she was listed for urgent liver transplantation. The anhepatic state was managed with intermittent plasmapheresis. She had transplantation 72 hours later, and was discharged from the hospital 3 weeks postoperatively after an uneventful recovery. She remains disease-free and has normal liver function 8 months after transplantation.

ALK-positive inflammatory myofibroblastic tumor of the abdomen with widespread microscopic multifocality.

Inflammatory myofibroblastic tumor (IMT) is a locally aggressive neoplasm, most frequently occurring in the abdominal cavity as multiple recurrent nodules. We report a case of IMT in a 24-year-old male presenting as multiple nodules involving the omentum, the liver, and the colon. Spindle tumor cells expressed ALK with a cytoplasmic granular distribution, the CLTC-ALK fusion gene was demonstrated by reverse-transcriptase polymerase chain reaction analysis, and break-apart fluorescence in situ hybridization (FISH) probes for the ALK gene showed a pathological pattern (single red signal associated with 1/2 normal fused signals) highly suggestive for combined gene fusion and deletion. To reduce the surgically unresectable liver mass, the patient was treated with crizotinib, and after 4 months of treatment the disease was defined stable according to RECIST criteria. Interestingly, ALK and FISH/FICTION analysis revealed that tumor cells were widely dispersed as multiple microscopic foci or as single cells beneath the omental mesothelium. These findings indicate that IMT multifocality might result either from dissemination from the main tumor mass or development of multiple independent neoplastic foci; furthermore, they underline the need of omentectomy in abdominal IMT to obtain surgical radicality.

Inflammatory myofibroblastic tumor of the appendix arising after treatment of gastric cancer: a case report and review of the literature.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue neoplasm of uncertain malignant potential and unclear etiology. IMT involving the appendix is very rare. Herein, we report a case of IMT of the appendix in a gastric cancer patient who was treated with radical gastrectomy and adjuvant systemic chemotherapy. Rare cases of IMT associated with preceding events have been described in other organs/sites, but not in the appendix. A previous intra-abdominal operation for gastric cancer may contribute to the development of IMT in the appendix as seen in the present patient. To our knowledge, this is the first case of appendiceal IMT arising after a previous operation.

Early and prolonged response to pazopanib in a patient with multiple metastases from renal cell carcinoma: a case report.

AIMS AND BACKGROUND: In recent years, targeted agents have replaced cytokine therapy as the standard of care for patients with metastatic renal cell carcinoma. METHODS AND STUDY DESIGN: We report a patient with multiple metastases from renal cell carcinoma treated with cytoreductive surgery and pazopanib. RESULTS AND CONCLUSIONS: The treatment resulted in an early and prolonged response, without toxicity.