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Indian Journal of Pathology &... 2022Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was...
BACKGROUND
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype.
DESIGN
A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia.
RESULTS
Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia.
CONCLUSION
Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.
Topics: Child; Humans; Anaplasia; Rhabdomyosarcoma; Sarcoma; Soft Tissue Neoplasms; Rhabdomyosarcoma, Alveolar
PubMed: 36308195
DOI: 10.4103/ijpm.ijpm_178_21 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Nov 2021Inflammatory myofibroblastoma (IMT) is a rare solid tumor, and its etiology and pathogenesis are unclear. Crohn's disease is a non-specific intestinal inflammatory...
Inflammatory myofibroblastoma (IMT) is a rare solid tumor, and its etiology and pathogenesis are unclear. Crohn's disease is a non-specific intestinal inflammatory disease. The clinical manifestations, laboratory examinations, and imaging examinations of IMT are not specific, making diagnosis difficult. A case of Crohn's disease combined with IMT of abdominal wall was admitted to the Department of Gastroenterology at the Third Xiangya Hospital, Central South University, on Nov. 21, 2017. This patient was admitted to our hospital because of repeated right lower abdominal pain for 4 years. A 6 cm×5 cm mass was palpated in the right lower abdomen. After completing the transanal double-balloon enteroscopy and computed tomographic enterography for the small intestinal, the cause was still unidentified. The patient underwent surgery due to an abdominal wall mass with intestinal fistula on Sept. 12, 2018 and recovered well currently. According to histopathology and immunohistochemistry, he was diagnosed with Crohn's disease combined with IMT. Up to July 2020, the patients still took azathioprine regularly, without abdominal pain, abdominal distension, and other discomfort, and the quality of his life was good.
Topics: Abdominal Pain; Abdominal Wall; Crohn Disease; Humans; Intestine, Small; Male; Neoplasms, Muscle Tissue
PubMed: 34911868
DOI: 10.11817/j.issn.1672-7347.2021.200966 -
International Journal of Surgical... Dec 2023Myofibroblastoma is a rare benign mesenchymal tumor first described in the breast. It is also known as mammary-type myofibroblastoma outside of the breast, more... (Review)
Review
Myofibroblastoma is a rare benign mesenchymal tumor first described in the breast. It is also known as mammary-type myofibroblastoma outside of the breast, more frequently located along the embryonic milk line. Exceptionally, myofibroblastoma can occur at visceral locations. We present a case of myofibroblastoma detected incidentally in the liver. A well-circumscribed mass, grossly measuring 6.2 cm in the liver parenchyma, was found on imaging studies. Histologically, the lesion is characterized by benign spindle cells in a hyalinized collagenous stroma, with positive staining for SMA and ER, focal positivity for CD34, negative for desmin, and loss of RB1. This rare tumor at such an unusual location makes it diagnostically challenging, especially on core biopsy of the lesion. To our knowledge, this is the second case of myofibroblastoma in the liver reported in the English literature and the first such case with a detailed pathology description.
Topics: Humans; Biomarkers, Tumor; Immunohistochemistry; Neoplasms, Muscle Tissue; Breast; Liver
PubMed: 36917841
DOI: 10.1177/10668969231160262 -
Journal of Nippon Medical School =... Jan 2021Angioleiomyomas typically present as small, painful, soft-tissue tumors less than 2 cm in diameter. The features of angioleiomyomas on magnetic resonance (MR) imaging...
BACKGROUND
Angioleiomyomas typically present as small, painful, soft-tissue tumors less than 2 cm in diameter. The features of angioleiomyomas on magnetic resonance (MR) imaging are not well understood, and the association of MR findings with histologic subtype is unclear. In the present study, the MR features of angioleiomyomas of average size were compared in relation to histologic subtype.
METHODS
This retrospective review of medical records analyzed MR imaging data and histologic specimens from 18 consecutive patients with angioleiomyomas that were resected at our hospital during the period from January 2006 through December 2013.
RESULTS
On T1-weighted images, lesions exhibited homogeneous areas that were isointense with skeletal muscle. However, T2-weighted images of solid and venous angioleiomyomas showed heterogeneous areas that were isointense or slightly hyperintense, while cavernous angioleiomyomas exhibited hyperintensity. Most lesions had a hypointense rim, and two thirds had adjacent vessels.
CONCLUSIONS
Our results suggest that MR findings for angioleiomyoma vary in relation to histologic subtype. T2-weighted images of solid and venous angioleiomyomas yielded specific MR findings that allowed for differentiation from other soft-tissue tumors, such as soft-tissue sarcomas. Most of these tumors exhibited isointense to slightly hyperintense regions, as compared with skeletal muscle, while findings for cavernous angioleiomyomas were nonspecific. Thus, clinical findings and MR imaging were almost sufficient for preoperative diagnosis of solid and venous angioleiomyomas.
Topics: Adult; Aged; Angiomyoma; Diffusion Magnetic Resonance Imaging; Female; Humans; Male; Middle Aged; Retrospective Studies; Soft Tissue Neoplasms
PubMed: 32238733
DOI: 10.1272/jnms.JNMS.2020_87-602 -
The American Journal of Pathology Nov 1949
Topics: Humans; Neoplasms; Neoplasms, Muscle Tissue
PubMed: 15392468
DOI: No ID Found -
Human Pathology Jun 2018Uterine smooth muscle tumors (USMTs) consist of a group of histologically heterogeneous and clinically diverse diseases ranging from malignant leiomyosarcoma (LMS) to... (Comparative Study)
Comparative Study
Uterine smooth muscle tumors (USMTs) consist of a group of histologically heterogeneous and clinically diverse diseases ranging from malignant leiomyosarcoma (LMS) to benign leiomyoma (ULM). The genetic alterations in LMS are complex, with some genetic alterations present in both LMS and other atypical histologic variants of USMT. In this study, we reviewed 119 USMTs with a diagnosis of LMS, smooth muscle tumor of uncertain malignant potential, atypical leiomyomas/leiomyoma with bizarre nuclei, and cellular leiomyoma, as well as 46 ULMs and 60 myometrial controls. We selected 17 biomarkers highly relevant to LMS in 4 tumorigenic pathways including steroid hormone receptors (estrogen receptor [ER] and progesterone receptor [PR]), cell cycle/tumor suppressor genes, AKT pathway markers, and associated oncogenes. ER and PR expression was significantly lower in LMS than smooth muscle tumor of uncertain malignant potential, atypical leiomyomas/leiomyoma with bizarre nuclei, cellular leiomyoma, and ULM (P < .01). Sixty-five percent of LMSs showed complete loss of ER, and 75% of LMSs showed complete loss of PR. All cell cycle genes were differentially expressed in different types of tumor, but significant overlap was noted. More than 75% of LMSs had Ki-67 index greater than 33%, and only 5% in all other types of USMT. Expression of the selected oncogenes varied widely among different types of USMT. PR positivity and p53 had a borderline association with progression-free survival (P = .055 for PR and P = .0847 for p53). Furthermore, high PR expression was significantly associated with a longer overall survival (P = .0163, hazard ratio 0.198). Cell proliferative indices (Ki-67) and sex steroid hormone receptors were the most valuable markers in differentiating LMS from other USMT variants.
Topics: Adult; Biomarkers, Tumor; Case-Control Studies; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Leiomyoma; Leiomyosarcoma; Middle Aged; Predictive Value of Tests; Progression-Free Survival; Time Factors; Tissue Array Analysis; Uterine Neoplasms
PubMed: 29258902
DOI: 10.1016/j.humpath.2017.12.005 -
BMJ Case Reports Nov 2023We present the case of a vulval superficial myofibroblastoma with a lymphocytic and eosinophilic rim in a woman in her late 20s. The tumour presented in pregnancy as a...
We present the case of a vulval superficial myofibroblastoma with a lymphocytic and eosinophilic rim in a woman in her late 20s. The tumour presented in pregnancy as a cystic lesion with pain and increasing size. While the histopathology of superficial myofibroblastomas has been well defined in the literature, to our knowledge, there has been no documentation of the presence of an inflammatory infiltrate of lymphocytes and eosinophils surrounding and within the tumour. This may potentially act as a diagnostic or prognostic reference.
Topics: Female; Humans; Eosinophilia; Eosinophils; Lymphocytes; Neoplasms, Muscle Tissue; Vulvar Neoplasms; Adult
PubMed: 38035683
DOI: 10.1136/bcr-2022-248800 -
Journal of Medical Case Reports Jan 2016Atypical uterine leiomyomas show benign behavior. However, the distinction between leiomyomas and leiomyosarcomas may at times be problematic. We report a rare case of... (Review)
Review
BACKGROUND
Atypical uterine leiomyomas show benign behavior. However, the distinction between leiomyomas and leiomyosarcomas may at times be problematic. We report a rare case of atypical uterine leiomyoma. We try to investigate potential immunohistochemical parameters that could be essential to distinguish cases of malignant smooth muscle tumors and those of uncertain or borderline histology.
CASE PRESENTATION
A 56-year-old white ethnic Albanian woman from Kosovo presented with uterine bleeding because of uterine multiple leiomyomas. A hysterectomy with unilateral adnexectomy was performed. Her hysterectomy specimen contained multiple leiomyomas in submucosal, intramural and subserosal locations. The leiomyomas were well demarcated, firm and white with a whorled cut surface and one had foci of hemorrhage. Histology of most of the leiomyomas showed a whorled (fascicular) pattern of smooth muscle bundles separated by well-vascularized connective tissue. Smooth muscle cells were elongated with eosinophilic or occasional fibrillar cytoplasm and distinct cell membranes. Some of them developed areas of degeneration including hyaline change, with less than five mitotic figures per ten high power fields in most mitotically active areas, and no significant atypia. One leiomyoma was characterized by moderately to severely pleomorphic atypical tumor cells with low mitotic counts and no coagulative tumor cell necrosis. Immunohistochemistry showed strong immunoreactivity for vimentin, smooth muscle actin and desmin, while cyclin-dependent kinase inhibitor 2A (p16), and B-cell lymphoma 2 (bcl-2) showed focal immunoreactivity, estrogen and progesterone were positive, Ki-67 expressed a low proliferation index, whereas p21 and tumor suppressor gene p53 were negative.
CONCLUSIONS
The combination of evaluation of conventional morphologic criteria with cyclin-dependent kinase inhibitor 2A (p16), p21, progesterone, B-cell lymphoma 2, tumor suppressor gene p53 and Ki-67 expression may be of great value in the assessment of uterine smooth muscle tumors of uncertain or borderline histology.
Topics: Diagnosis, Differential; Female; Humans; Leiomyomatosis; Leiomyosarcoma; Middle Aged; Mitotic Index; Uterine Neoplasms
PubMed: 26801982
DOI: 10.1186/s13256-016-0800-3 -
Cirugia Y Cirujanos 2017Intussusception is defined as a segment of the gastrointestinal tract and mesentery within the lumen of an adjacent segment. It is a rare condition in adults that can... (Review)
Review
BACKGROUND
Intussusception is defined as a segment of the gastrointestinal tract and mesentery within the lumen of an adjacent segment. It is a rare condition in adults that can occur anywhere in the gastrointestinal tract from the stomach to the rectum. Only 5% of all intussusceptions are presented in adults, and in 1-5% of all cases of intestinal obstruction. Inflammatory myofibroblastic tumour is rare, and is usually found in the lung, and rarely detected in some intestinal portions. It causes a variety of non-specific symptoms, with those that present as an intussusception being uncommon.
CLINICAL CASE
A female of 69 years with partial bowel obstruction secondary to intestinal intussusception due to an inflammatory myofibroblastic tumour, a rarely diagnosed condition and never published before.
DISCUSSION
Inflammatory myofibroblastic tumours are rare, and in this case with an atypical presentation that was surgically resolved satisfactorily. These entities are difficult to diagnose, with histopathology giving the definitive diagnosis. A literature review was performed to gather recent information about their diagnosis and treatment.
CONCLUSIONS
Inflammatory myofibroblastic tumours require a high level of suspicion, as diagnosis prior to surgery is difficult. Surgery is considered the treatment of choice, requiring leaving free surgical edges to prevent recurrences.
Topics: Abdomen, Acute; Aged; Female; Humans; Ileal Diseases; Ileal Neoplasms; Intussusception; Ischemia; Neoplasms, Muscle Tissue
PubMed: 27568400
DOI: 10.1016/j.circir.2016.06.001 -
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