Inflammatory myofibroblastic tumor: A demographic, clinical and therapeutic study of 92 cases.

PURPOSE: Inflammatory myofibroblastic tumors (IMT) was a rare kind of tumor defined by WHO since 2012. Little was known about this disease. There were controversies about IMT's behavior, predilection site, age distribution, and the best treatment methods. Here we provided a systematic overview on tumor demographical, clinical, biological features as well as treatment efficacy based on real cases from Surveillance, Epidemiology, and End Results (SEER) database. METHODS: 92 patients diagnosed with IMT by histopathology were drawn from SEER database between 2002 and 2014. Patient demographics, clinical features and treatment information were analyzed. RESULTS: The mean age of onset was 47.4 ± 22.4 years (0 to 83y) and the ages prone to this disease are middle-aged (from 41y to 64y), accounting for 1/3 of all patients. Three peak ages of onsets were 0-4y, 36-40y and more than 50y. 42% of the tumors were located in the soft tissues of limbs, hip, shoulder, head, face and neck. The average tumor sizes were 6.5 ± 5.3cm (1cm to 25cm). Survival in the group of tumor size smaller than 6.5cm was better compared to group of tumor size larger than 6.5cm (P < 0.05). Most of the tumors were malignant or malignant potential (89%), though local and distant metastasis rate were low (5%). Surgery was the most common treatment. However, the survival benefit was still uncertain compared to adjuvant chemotherapy or radiotherapy. Multivariate regression analysis demonstrated that young patients had better survival than old ones. CONCLUSIONS: IMT was a malignant tumor with low risk of local and distant metastasis. The peak ages were 0-4y, 36-40y and more than 50y. The prone sites were the soft tissues of the limbs, hip, shoulder, head, face and neck. Tumor sizes and ages were the factors correlated with survival time.

Uterine inflammatory myofibroblastic tumor: more common than expected: Case report and review.

RATIONALE: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm composed of spindled to epithelioid cells with prominent myxoid stroma and inflammatory infiltrate. It has a low but definite malignant potential. However, its management has never been standardized. PATIENT CONCERNS AND DIAGNOSIS: We present the first case of uterine IMT laparoscopically treated. Moreover, we reviewed the English literature regarding uterine IMT published between 1987 and June 2017. A total of 72 cases of uterine IMT were included. Clinical and pathological characteristics, treatments and outcomes were recorded. INTERVENTIONS AND OUTCOMES: A total laparoscopic hysterectomy with opportunistic bilateral salpingectomy was performed. Patient is free of disease at 6 months of follow-up. LESSONS: Uterine IMT may be identified by anaplastic lymphoma kinase overexpression, its prognosis is usually good, complete excision seems to be effective to avoid relapse and mini invasive surgery seems to be effective and safe to treat uterine IMT. However, considering the age of women affected by disease, conservative management, or medical therapy could be taken in account to avoid surgical injuries and to preserve fertility.

Management of musculoskeletal tumors during pregnancy: a retrospective study.

BACKGROUND: In recent years, scientific research has increasingly focused on malignancies during pregnancy. However, the development of musculoskeletal tumors during pregnancy has only been the subject of a few studies so far. The primary aim of this study was to identify the incidence of sarcomas during pregnancy at our musculoskeletal tumor center (MSTC). Secondarily we intended to analyze these cases and discuss possible recommendations regarding diagnostic work-up as well as therapy on the basis of the literature. METHODS: All female patients who had been treated for soft tissue or bone sarcoma at our academic MSTC in the period between the years 2002 and 2010 were screened retrospectively for anamnestic annotations of pregnancy or records of pregnancy in the obstetrical database of our university hospital. The patients who met the criteria for inclusion (diagnosed sarcoma and pregnancy) were enrolled. For every pregnant patient two age-matched female control patients that suffered from tumors with the same histologic type were included. RESULTS: In the period between 2002 and 2010, 240 female patients between the age of 16 and 45 were treated for sarcoma. In eight out of the 240 cases the tumor disease developed or progressed during pregnancy. The delay in diagnosis was approximately eight months and turned out to be significantly higher for pregnant patients compared to non- pregnant controls. Each woman's tumor was misdiagnosed at least once. CONCLUSIONS: Diagnostic follow-up of pregnant women presenting with a growing or painful mass, which is suspected to be a musculoskeletal tumor, should be performed at a specialized tumor center. We recommend a multidisciplinary approach and discussing all possible consequences for mother and child intensively in accordance with the available literature.

Inflammatory myofibroblastic tumour of the bladder in children: a review.

INTRODUCTION: Inflammatory myofibroblastic tumours of the bladder (IMTB) are rare, and feature a benign and reactive proliferation of myofibroblasts. 25% of the reported IMTB cases in the literature occur in children. The present study presents a review of IMTB in children. DISCUSSION: The data from 42 reported cases of paediatric IMTB in the world literature are summarised, including two recent cases from the present centre. Paediatric IMTB equally affects males and females. It mainly presents with haematuria, dysuria or abdominal pain. Lesions can vary in size, but mean size is 5.5 cm. Mean age is 7.5 years. The aetiology of IMTB is poorly understood, but includes infective or traumatic aetiologies, or a possible clonal lesion. IMTB may specifically show clonal gene rearrangements involving the anaplastic lymphoma kinase (ALK-1) gene. To differentiate IMTB from rhabdomyosarcoma, tissue diagnosis and careful histological analysis are essential. Tumour biopsy can be achieved by a transurethral approach or a transcutaneous approach with ultrasound guidance. Between 35 and 89% of cases of IMTB express ALK-1 by immunohistochemistry. ALK-1 expression is much less common in other bladder soft tissue tumours. ALK-1 is thus useful in the diagnosis of IMTB. The treatment of choice for IMTB is complete surgical resection of the lesion. In children, no proven recurrent or metastatic IMTB episodes are reported after excision. However IMTB recurrences are reported in adults, likely due to incomplete excision. Follow-up after excision is therefore recommended. CONCLUSIONS: Paediatric IMTB is uncommon. Tissue biopsy is essential for diagnosis. Careful histological assessment is required to differentiate IMTB from malignant paediatric bladder tumours such as rhabdomyosarcoma. ALK-1 expression is useful in confirming the diagnosis of IMTB. Treatment of choice is complete surgical resection of the lesion. Recurrence is reported in adult IMTB. Follow-up is therefore recommended.

[Clinical analysis of 14 patients with extraorbital inflammatory myofibroblastic tumor of the head and neck].

OBJECTIVE: To summarize the clinical features of extraorbital inflammatory myofibroblastic tumor (IMT) of the head and neck. METHODS: Fourteen cases of extraorbital IMT treated in recent 20 years were analyzed retrospectively. RESULTS: Of the 14 patients, 9 cases with limited lesion in maxilla (n = 5), mandible (n = 2) or neck (n = 2) underwent local resection, and no recurrences were found after 1.5 to 20.0 years; 3 cases diagnosed as maxillary IMT involved in orbit, hard palate or pterygopalatine fossa received conservative therapy (prednisone, prednisone plus radiotherapy or prednisone plus chemotherapy), and no disease progression was found after 6, 9 or 2 years respectively; and 1 case diagnosed as maxillary IMT involved in orbit and pterygopalatine fossa was confirmed with cervical metastases after two operations and died of brain invasion within 17 months. One patient with localized lesion around the common carotid artery was treated with prednisone and had no disease progression with a 2-year follow-up. CONCLUSIONS: Extraorbital IMT of the head and neck is a rare clinical entity. Pathology examination is required for final diagnosis. Corticosteroid administration may be a choice of treatments, and radical resection should be taken selectively for limited lesions.

Soft-tissue inflammatory myofibroblastic tumors (IMTs) of the limbs: potential and limits of diagnostic imaging.

OBJECTIVE: The objective of this work was to evaluate the potential of diagnostic imaging in the identification, localization, and characterization of soft-tissue inflammatory myofibroblastic tumors (IMTs) of limbs with correlation to differential diagnosis and therapy. MATERIALS AND METHODS: From a retrospective analysis of 324 histologically verified soft-tissue lesions of limbs and extremities diagnosed in our institute from January 2002 to July 2010, we selected seven cases of histologically proven IMT. These included six males and one female, aged between 28 and 81 years (mean age, 57 years). Lesions were localized in three cases to the thigh, in two cases to the popliteal space, and in the remaining two cases, to the shoulder girdle. All patients were evaluated on the basis of US, CT, and MRI. RESULTS: Ultrasound detected the presence of a non-homogeneous solid formation in all cases and calcifications in three cases. CT showed the presence and type of calcification/ossification and bone reaction. On MRI, all cases had low signal intensity on SE T1-weighted sequences and an intermediate-low signal intensity on SE and FSE T2-weighted sequences in six of them; only one case had an intermediate-high signal intensity on SE and FSE T2-weighted sequences. Both contrast-enhanced CT and MRI showed precocious enhancement in association with multiple peripheral hypertrophic blood vessels. CONCLUSIONS: On the basis of integrated imaging data obtained by US, CT, and MRI, it is possible to evaluate the lesion extension to provide a loco-regional staging, to characterize IMTs, and to allow an optimal therapeutical planning.

Abdominal inflammatory myofibroblastic tumor a clinicopathologic study with reappraisal of biologic behavior.

BACKGROUND AND PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a proliferative lesion of controversial nosology and uncertain prognosis. In an attempt to acquire further understanding of pathogenesis and biologic behavior, we surveyed abdominal IMTs managed over the last 12 years at a single institution. METHODS: Intra-abdominal IMTs treated between 1995 and 2007 were reviewed concerning demographic, clinical, and pathologic features as well as therapeutic management and outcome. All specimens were reevaluated by histologic examination and immunohistochemistry. RESULTS: There were 7 patients (4 males; age range, 28 days to 14 years). Five lesions were located in alimentary tract: 1 gastric presenting with bleeding, 1 hepatic presenting with a thoracic wall mass, 1 pancreatic and 2 colonic presenting with obstructive symptoms. One splenic IMT was found incidentally. The remaining case arose from the adrenal gland and presented with a palpable mass. The gastric and adrenal IMTs had evidence of a previous or concomitant infectious setting. Five lesions were excised. The pancreatic IMT underwent a drainage procedure followed by steroid administration, and the hepatic lesion received antibiotics. Histopathology revealed characteristic findings of IMT. Expression of anaplastic lymphoma kinase was negative in all cases. At a median follow-up of 6 years (range, 3-15), all children were asymptomatic with no recurrences. The hepatic and pancreatic IMT displayed complete and near total regression, respectively. CONCLUSION: A benign behavior of abdominal IMTs was observed even in patients not undergoing surgical excision. Although IMT remains a surgical disease, a conservative approach may be reasonable in select cases.

Vascular smooth muscle tumors: review of the literature.

Vascular smooth muscle tumors are very rare. They can be benign or malign. Intravascular leiomyomatosis is a benign neoplasm that extends through the veins and caries significant morbidity. Angioleiomyoma is a benign neoplasm of the extremities that caries minimal morbidity. Vascular leiomyosarcomas are malign neoplasms derived from vascular smooth cells. They are usually localized to the inferior vena cava, but can also arise from the pulmonary arteries or veins or other peripheral vessels. This study reviews literature for epidemiology, clinical presentation, diagnosis and management of patients with vascular smooth muscle tumors.

Mesenchymal tumors of the mouse urinary bladder with vascular and smooth muscle differentiation.

Bifenthrin, a synthetic pyrethroid insecticide/miticide, has been fed to male and female Swiss Webster mice at levels of 0, 50, 200, 500, and 600 ppm in the diet for between 604 and 644 days. Tumors of the urinary bladder were observed and initially reported as leiomyosarcomas. Subsequently, the bladders were reviewed and the tumors showed a pattern of both epithelioid cells and spindle cells forming irregular vascular channels. The tumors appeared to arise from the trigone of the bladder and, in some cases, invaded the bladder wall. No metastases were recorded. The tumor is usually considered rare; however, in this study, it was commonly observed in all groups but predominantly in males. The histogenesis of the tumor is uncertain, but from its pleomorphic histological features, including smooth muscle and vascularity, it is probably derived from vascular mesenchyme.

Smooth muscle tumors of the appendix and colon: a collective review of the world literature.

In this collective review, we compiled all reported cases of smooth muscle tumors of the colon/appendix in the world literature from 1959 to 1989. Our goal was to make more accurate conclusions about these tumors based on our increased data pool.

Smooth muscle tumors of the rectum and anus: a collective review of the world literature.

In this collective review, we have compiled all the reported cases of smooth muscle tumors of the rectum/anus in the world literature from 1959 to 1989. Our goal was to increase the data pool of smooth muscle tumors by adding these new data to that previously collected from 1881 to 1959. We increased the pool for leiomyomas from 89 to 148 and that for leiomyosarcomas from 54 to 215. By doing this, we hoped to make more accurate conclusions about smooth muscle tumors based on this increased data pool. Some interesting findings included three cases in small children that were found in our recent review: a 2-year-old with a leiomyoma and two small infants, aged 12 days and 36 days, with leiomyosarcomas. Again, the findings were probably consistent with an increased data pool. We were also able to find several more cases involving the anal region. We found the highest incidence of leiomyomas to have increased by a decade from the 40-49 year age group to the 50-59 year age group, while among leiomyosarcomas, there was about equal incidence among the 50-59 and 60-69 age groups. We doubt that these represent actual changes in the demographics, but rather that these latter findings are more accurate based on the greater quantity of cases available to us. As a further example, we found no appreciable sex difference; however, we did find more cases reported in females. From our increased data pool, we were able to find 16 more cases that were described as dumbbell-shaped, compared to one that was reported before 1959. Palpable mass, hemorrhage, and pain/discomfort continued as the most common symptoms reported at presentation. With regard to size, the majority of leiomyomas were found to be less than 5 cm in diameter, closely followed by those 5-9 cm. The majority of leiomyosarcomas were 5-9 centimeters at discovery. Most cases of leiomyoma were treated by excision, while most cases of leiomyosarcoma were treated by abdominoperineal resection, a finding consistent with old data. We hope that this paper thoroughly reviews pertinent information about leiomyomas and leiomyosarcomas of the rectum/anus and, in doing so, serves to refresh a few memories, stimulate others, and teach a few.

Granular cell tumors of the head and neck in children: the experience at the Children's Hospital of Philadelphia.

Granular cell tumors are rare, usually benign tumors of controversial histogenesis. These tumors are uncommon in children. Granular cell tumors in adults have a predilection for the head and neck, but this was not noted in our experience with children. Of the 22 granular cell tumors diagnosed and managed at the Children's Hospital of Philadelphia from 1960 to 1988, 7 were from the head and neck. One child with a subglottic granular cell tumor is of particular interest and is presented in detail.

Congenital smooth muscle hamartoma. Prevalence, clinical findings, and follow-up in 15 patients.

Cogenital smooth muscle hamartoma is a congenital skin lesion characterized by proliferation of bundles of smooth muscle within the reticular dermis. We report on a group of 15 children with this lesion, confirmed by skin biopsy specimen, its prevalence, the occurrence of associated anomalies, and long- term follow-up. To our knowledge, this is the largest group of patients with this lesion described in the literature. The estimated prevalence is about 1:2600 live births with slight male predominance. The lesion is most frequent in the lumbosacral area (67% [10/15]) and a positive pseudo-Darier's sign is present in 80% (12/15) of patients. During the follow-up period of up to 7 years, the lesions enlarged slightly but became less prominent. No malignant transformation was observed.

Intrathoracic granular cell myoblastoma.

Intrathoracic granular cell myoblastoma (GCM) is most commonly a benign neoplasm of Schwann cell origin that occurs in the large airways, the segmental bronchi, and rarely in the mediastinum. The tumor predominantly affects blacks during the fourth decade, and shows no sex predilection. Tracheobronchial GCM most commonly produces cough and chest pain, with focal consolidation or atelectasis shown on chest x-ray films. Extraesophageal mediastinal GCM produces few or no symptoms, and should be included in the differential diagnosis of a posterior mediastinal mass. Computerized tomography is helpful in evaluating the extent of the lesion, defining its relationship to adjacent structures, and planning the therapeutic approach. The diagnosis of tracheobronchial GCM usually can be made by bronchoscopy and/or biopsy. Treatment remains controversial, but most studies suggest surgical or endoscopic resection as the treatment of choice. The prognosis of intrathoracic GCM is good but is dependent upon complete removal.

Granular cell tumors of the head and neck.

Granular cell tumors have a predilection to occur in the head and neck. Thirty granular cell tumors have been diagnosed during the past 26 years at UCLA; 13 of which presented in the head and neck. Of these 13 cases, 10 were correctly diagnosed on the primary pathological specimen, which included one fine-needle aspirate and three frozen sections. Four cases required diagnostic confirmation with electron microscopy or histochemistry. The tongue accounted for more than one third of the cases. Three lesions exhibited locally aggressive behavior, but none of the tumors metastasized. Initial treatment was wide local excision in all but one case; recurrence was noted in two cases. Fine-needle aspiration can be used to provide preoperative diagnosis of granular cell tumors. Wide local excision with histologically confirmed clear margins provides definitive treatment.

Granular cell tumours of the vulva.

Granular cell tumours occur in a variety of sites, including the vulva. Origins from myogenic, histiocytic, fibroblastic and neurogenic elements have been proposed. Female preponderance suggests that oestrogenic hormones are involved. Seven cases of granular cell tumours of the vulva have been studied. In none was the correct diagnosis made preoperatively. They were solitary lesions and local excision was curative. Paraffin sections of these cases were studied by peroxidase-antiperoxidase method for myoglobin, lysozyme, alpha-1-antitrypsin and S-100 protein localization. Antimyoglobin, antilysozyme and anti-alpha-1-antitrypsin antibodies were not localized in these tumours; however, S-100 protein was localized in all of them. These results agree with previous data that suggest a neurogenic origin for granular cell tumours.