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Journal of Cardiothoracic Surgery Aug 2020Angioleiomyoma of the pulmonary artery is rare in the literature and few studies have been reported. Here we present a rare case of angioleiomyoma arising from the... (Review)
Review
BACKGROUND
Angioleiomyoma of the pulmonary artery is rare in the literature and few studies have been reported. Here we present a rare case of angioleiomyoma arising from the pulmonary artery in a young patient.
CASE PRESENTATION
A 27-year-old male patient presented to our clinic due to the incidental finding of a nodule in the right lower lobe of the lung, which was unchanged from the prior year. Preoperative CT scans showed a well-demarcated nodule of soft tissue density penetrated by the basal branch of the right anterior basilar artery (RA8b). Single-port video-assisted RS8 segmentectomy was performed under the guidance of preoperative 3-dimensional reconstruction for histologic confirmation of the tumour. The tumour appeared as a solid tumour of a tube-like structure with vascular endothelium, composed of spindle-shaped smooth muscle cells lacking nuclear atypia and homogenous red-dye substances. The spindle cells were positive for immunostaining for smooth muscle actin (SMA), desmin and Ki-67 and were negative for immunostaining for Dog-1, HMB45, and Melan-A. A pathological diagnosis of primary angioleiomyoma of the pulmonary artery was finally made.
CONCLUSIONS
This report is a reminder for thoracic surgeons that angioleiomyoma should be included in the differential diagnosis of lung neoplasms, especially for the mass of soft tissue density penetrated by pulmonary blood vessels shown by CT. Awareness of this rare entity should potentially prevent underdiagnosis and improper surgical treatment.
Topics: Adult; Angiomyoma; Diagnosis, Differential; Humans; Lung Neoplasms; Male; Pulmonary Artery; Tomography, X-Ray Computed; Vascular Neoplasms
PubMed: 32859240
DOI: 10.1186/s13019-020-01275-z -
International Journal of Radiation... Jan 2019For infants with localized rhabdomyosarcoma who were enrolled on Children's Oncology Group ARST0331 and ARST0531, local therapy guidelines were provided, but adherence...
PURPOSE
For infants with localized rhabdomyosarcoma who were enrolled on Children's Oncology Group ARST0331 and ARST0531, local therapy guidelines were provided, but adherence was not mandated because of toxicity concerns. We examined adherence to protocol for local therapy guidelines, treatment variations, and outcomes for these patients.
METHODS
Children aged ≤24 months who were enrolled on ARST0331 and ARST0531 were evaluated. Data were verified through radiologic, surgical, pathologic, and clinical records. Local therapy was assessed for adherence to protocol guidelines, with variations termed "individualized local therapy." The subdistribution hazards model assessed local failure, the Kaplan-Meier product limit method assessed event-free survival (EFS) and overall survival, and the log-rank test was used to evaluate prognostic impact.
RESULTS
The median age of the patients was 14 months, and 124 patients were eligible. Common primary sites were genitourinary (40%), parameningeal (14%), and the extremities (11%). Most patients had unresected disease (group 3, 64%) and embryonal histology (73%). Fifty-eight percent of patients received radiation therapy at a median of week 12 (weeks 1-45). The median radiation dose was 48.6 Gy (30.6-54 Gy). Forty-three percent of patients received individualized local therapy (outside protocol guidelines), typically omission or delay of radiation therapy. Delayed primary excision was performed in 28% at a median of week 14 (weeks 7-34). With a median follow-up of 5.6 years, the 5-year local failure, EFS, and overall survival rates were 24%, 68%, and 82%, respectively. Local failure was significantly higher (35%) in patients receiving individualized local therapy than in patients who received protocol-specified local therapy (16%; P = .02). The site of failure was local in 64% of patients, local and distant in 5%, and distant only in 23%. EFS was significantly higher among patients who were aged 12 to 24 months, had tumors ≤5 cm, had group 1/2 disease, and underwent protocol-specified therapy.
CONCLUSIONS
Local recurrence was the predominant pattern of failure and was more common in those receiving individualized local therapy. De-escalation of effective therapies because of concerns about treatment toxicity should be considered cautiously.
Topics: Female; Guideline Adherence; Humans; Infant; Infant, Newborn; Male; Radiotherapy Dosage; Rhabdomyosarcoma
PubMed: 30138647
DOI: 10.1016/j.ijrobp.2018.08.017 -
Gastroenterologie Clinique Et Biologique May 2001
Review
Topics: Female; Gastrointestinal Neoplasms; Humans; Immunohistochemistry; Male; Microscopy, Electron; Neoplasms, Muscle Tissue; Prognosis; Racial Groups
PubMed: 11521101
DOI: No ID Found -
Journal of Veterinary Diagnostic... Nov 2019A large, ill-defined, firm, multinodular mass involving the pancreas was confirmed on postmortem examination of a 5-y-old, male Rottweiler that died following acute...
A large, ill-defined, firm, multinodular mass involving the pancreas was confirmed on postmortem examination of a 5-y-old, male Rottweiler that died following acute respiratory distress syndrome, after a period of anorexia and lethargy. Histologically, the mass consisted of plump spindle cells admixed with a variable number of macrophages, lymphocytes, plasma cells, and neutrophils. Foci of coagulative necrosis and hemorrhage were also observed. Spindle cells strongly reacted to antibodies against vimentin, α-smooth muscle actin, and calponin, whereas desmin was expressed only mildly and focally. Pan-cytokeratin, KIT, glial fibrillary acidic protein, and S100 protein were nonreactive. Variable numbers of MAC 387-positive cells, CD3+ lymphocytes, and numerous blood vessels were also detected throughout the mass. Histologic and IHC findings were consistent with a diagnosis of inflammatory myofibroblastic tumor of the pancreas.
Topics: Animals; Dog Diseases; Dogs; Fatal Outcome; Inflammation; Male; Neoplasms, Muscle Tissue; Pancreatic Neoplasms
PubMed: 31585512
DOI: 10.1177/1040638719879737 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Dec 2023Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with...
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.
Topics: Humans; Infant; Granuloma, Plasma Cell; Nasopharynx; Neoplasms, Muscle Tissue; Soft Tissue Neoplasms
PubMed: 38114319
DOI: 10.13201/j.issn.2096-7993.2023.12.013 -
Pathology Oncology Research : POR Sep 2008Myogenin immunostaining has been described as a useful marker of the alveolar subtype of rhabdomyosarcoma and as a tool for distinguishing it from the more common... (Review)
Review
Myogenin immunostaining has been described as a useful marker of the alveolar subtype of rhabdomyosarcoma and as a tool for distinguishing it from the more common embryonal subtype. To add to the growing body of literature describing this phenomenon we analysed myogenin immunohistochemical staining in 152 tumors using a rhabdomyosarcoma tissue array. Results were analysed blinded to histological type by two independent investigators. Samples were excluded if any samples failed to stain with desmin and/or myogenin. Mean percentage of myogenin positive cells was significantly greater for ARMS (n = 31; mean percentage positivity 59% (95% confidence intervals +/- 7%) than ERMS (n = 41, mean percentage positivity 16%, 95% confidence intervals +/- 4; P < 0.0001). This data is consistent with previously published studies identifying strong nuclear myogenin staining in a high proportion of cells as a marker of alveolar histology.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Humans; Myogenin; Prognosis; Rhabdomyosarcoma, Alveolar; Rhabdomyosarcoma, Embryonal; Sensitivity and Specificity; Tissue Array Analysis
PubMed: 18493875
DOI: 10.1007/s12253-008-9012-5 -
Diseases of Aquatic Organisms Apr 2016A wild adult male conger Conger conger, captured by a SCUBA diver in the waters of coastal Italy, was sent for laboratory analysis due to the presence of multiple...
A wild adult male conger Conger conger, captured by a SCUBA diver in the waters of coastal Italy, was sent for laboratory analysis due to the presence of multiple productive ulcerous skin lesions localized in the dorso-lateral body area, caudally to the gill operculum under the dorsal fin. The main mass was sessile, ulcerated and hemorrhaging in appearance and was surrounded by several smaller masses, which originated peripherally from the same mass or were isolated, always with a tendency to ulceration. Histology confirmed that the newly formed tissue originated from derma invading the closer tissues. The tumour consisted of spindle cells, each with an ovoidal nucleus and rarely with evident nucleoli, which were arranged in parallel or storiform patterns and were often surrounding blood-filled spaces discontinuously lined by endothelial cells. Tumour tissue was richly vascularized and no mitoses were seen. The overlying epidermis was ulcerated. Masson's trichrome technique indicated the presence of a small amount of perivascular connective tissue. No excessive glycogen storage, bacteria, virus or fungi were detected by periodic acid-Schiff (PAS)-reaction. Immunohistochemistry showed dot-like or diffuse cytoplasmic positivity against smooth muscle actin and the monoclonal antibody D2-40. CD34 exhibited relevant immunoreactivity at plasma membranes. Growth fraction evaluated using MIB-1 was <1%. Immunoreactions for wide spectrum CK, CK5/6, CK8, CK18, EMA, desmin, myoglobin, S-100, CD20, CD68, GFAP, and NSE were negative. Histopathological and immunohistochemical results supported a diagnosis of angioleiomyoma, a benign tumour of the muscular cellular component of the blood vessels. To our knowledge, this is the first report of such neoplasms in fishes in which monoclonal antibodies work on fish tissues, facilitating a useful immunohistochemical approach for differential diagnosis.
Topics: Angiomyoma; Animals; Eels; Fish Diseases; Male; Skin
PubMed: 27068506
DOI: 10.3354/dao02984 -
Annals of the Royal College of Surgeons... Jan 2007In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy. Total... (Review)
Review
In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy. Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver. A 5-year survival of 70% can be achieved in such cases. In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation. An exception is those children in whom the hepatocellular carcinoma is detected during surveillance of chronic liver disease - they typically have smaller tumours and frequently have a good prognosis after liver transplantation. The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited. Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.
Topics: Carcinoma, Hepatocellular; Child; Hamartoma; Hemangioendothelioma, Epithelioid; Hepatoblastoma; Humans; Liver Diseases; Liver Neoplasms; Liver Transplantation; Neoplasms, Muscle Tissue; Sarcoma; Survival Analysis; Treatment Outcome
PubMed: 17316514
DOI: 10.1308/003588407X155527 -
Journal of Medical Case Reports Jun 2016This case report is interesting as cases of children with laryngeal inflammatory myofibroblastic tumor are not common and previously had been presented as isolated case... (Review)
Review
BACKGROUND
This case report is interesting as cases of children with laryngeal inflammatory myofibroblastic tumor are not common and previously had been presented as isolated case reports. This is the first case report in Asia describing a laryngeal inflammatory myofibroblastic tumor and its removal using an endoscopic approach.
CASE PRESENTATION
Our patient is a 12-year-old Malay girl from Singapore who presented with hoarseness without respiratory distress. The initial impression was that of a granuloma or a papilloma. We did a biopsy, which confirmed the histology to be inflammatory myofibroblastic tumor, and a magnetic resonance imaging scan showed a contrast-enhanced lesion. The lesion was excised completely using an endoscopic approach. The child was discharged well on the first postoperative day and she has been on follow-up for a year in the clinic.
CONCLUSIONS
This report highlights the importance of understanding the differential diagnosis for a child with hoarseness. It is not uncommon for a pediatrician, a general practitioner, and a pediatric otolaryngologist to see a child presenting with hoarseness. In most cases, the diagnosis made would be screamer's nodules, which is commonly seen in children. In a small group, recurrent respiratory papillomatosis form the diagnosis. Over the past few years, the cases of recurrent respiratory papillomatosis have decreased significantly. Laryngeal tumors are not common in children. However, we must maintain a high index of suspicion when we have a child with hoarseness who does not improve with speech therapy and watchful waiting. In such situations, a stroboscope is usually necessary to diagnose the voice problems and to rule out pathological conditions such as laryngeal tumors. If left untreated, the lesion can grow with time and result in a life-threatening airway condition. We also demonstrate our endoscopic technique in this report, and it has proven to be safe with no increased recurrence and much lower morbidity.
Topics: Biopsy; Bronchoscopy; Child; Female; Granuloma, Plasma Cell; Hoarseness; Humans; Immunohistochemistry; Laryngeal Diseases; Laryngoscopy; Larynx; Magnetic Resonance Imaging; Myofibroblasts; Neoplasms, Muscle Tissue; Treatment Outcome
PubMed: 27339455
DOI: 10.1186/s13256-016-0967-7 -
Journal of Medical Case Reports Jun 2015Mammary-type myofibroblastoma of the soft tissue is a very rare, benign, mesenchymal neoplasm with myofibroblastic differentiation. To date, 20 cases of extra-mammary... (Review)
Review
INTRODUCTION
Mammary-type myofibroblastoma of the soft tissue is a very rare, benign, mesenchymal neoplasm with myofibroblastic differentiation. To date, 20 cases of extra-mammary myofibroblastoma have been described in literature. To the best of our knowledge, this is the largest extra-mammary myofibroblastoma described in the literature, and the first case reported in this location.
CASE PRESENTATION
A 50-year-old Pakistani man presented with a long history of a painless, huge lump on his right thigh. His clinical examination showed normal-looking skin and there was no inguinal lymphadenopathy. The mass was excised with a clinical impression of soft tissue sarcoma. Gross examination showed a huge, well-circumscribed soft tissue mass measuring 34 cm in its largest dimension and weighing approximately 13 kg. It was partially covered by fat tissue. Histologically, the lesion was composed of a haphazard arrangement of bland spindle-shaped cell fascicles in a thick collagenous and myxoid background. The neoplastic cells showed diffuse and patchy positivity for CD34 and desmin, respectively. No recurrence was seen following surgical excision over a follow-up period of five months.
CONCLUSIONS
Mammary-type myofibroblastoma of the soft tissue is a benign soft tissue neoplasm, and no malignant behavior and/or recurrence after surgical resection has been described, regarding its size and location. As an extremely rare tumor, the correct diagnosis and prompt management is important, and requires careful clinical and pathological workup to rule out the possibility of a malignant neoplasm.
Topics: Humans; Male; Middle Aged; Neoplasms, Muscle Tissue; Soft Tissue Neoplasms; Thigh
PubMed: 26033228
DOI: 10.1186/s13256-015-0601-0