Abstract:Inflammatory myofibroblastic tumors are rare, and those located in the extremities without bone involvement are even rarer. We present the case of a 61-year-old Chinese male patient with an inflammatory myofibroblastic tumor of the right thigh. It was excised and a histopathologic examination revealed an inflammatory myofibroblastic tumor. This case is presented by virtue of its rare location.
“…IMT occurs in any anatomical part of the body. However, it rarely occurs in the soft tissues of the extremities, with no more than 20 cases reported, among which the thigh is the most common place, [2][3][4][5] followed by hand, 3,6 foot, 3 articular fossa, 7 and arm. 8 The age of IMT arising from the soft tissue of the extremities is widely distributed, ranging from 6 months to 61 years old.…”
A 34-year-old Chinese woman found a lump in her left leg for more than 3 weeks without any discomfort. Grossly, the tumor was relatively well delineated with focal infiltration. Histopathologic evaluation showed a compact fascicular spindle cell proliferation with variable myxoid and collagenous stroma and scattered inflammatory infiltrate. Immunohistochemically, the tumor cells showed positive expression of ALKD5F3 and SMA and negative expression of CD34, desmin, and cytokeretin. Fluorescence in situ hybridization analysis of the ALK locus showed break-apart signals in 20% of tumor cells, and DNA sequencing discovered a novel CLIP2-ALK fusion gene. The lesion was diagnosed as an inflammatory myofibroblastic tumor (IMT). To the best of our knowledge, this is the first case with CLIP2-ALK gene fusion in the somatic soft tissue IMTs.
“…IMT occurs in any anatomical part of the body. However, it rarely occurs in the soft tissues of the extremities, with no more than 20 cases reported, among which the thigh is the most common place, [2][3][4][5] followed by hand, 3,6 foot, 3 articular fossa, 7 and arm. 8 The age of IMT arising from the soft tissue of the extremities is widely distributed, ranging from 6 months to 61 years old.…”
A 34-year-old Chinese woman found a lump in her left leg for more than 3 weeks without any discomfort. Grossly, the tumor was relatively well delineated with focal infiltration. Histopathologic evaluation showed a compact fascicular spindle cell proliferation with variable myxoid and collagenous stroma and scattered inflammatory infiltrate. Immunohistochemically, the tumor cells showed positive expression of ALKD5F3 and SMA and negative expression of CD34, desmin, and cytokeretin. Fluorescence in situ hybridization analysis of the ALK locus showed break-apart signals in 20% of tumor cells, and DNA sequencing discovered a novel CLIP2-ALK fusion gene. The lesion was diagnosed as an inflammatory myofibroblastic tumor (IMT). To the best of our knowledge, this is the first case with CLIP2-ALK gene fusion in the somatic soft tissue IMTs.
“…Patients with IMTs of the extremities have no systemic symptoms; anemia, fever, or weight loss, and laboratory abnormalities are uncommon [ 13 ]. However our patient had high levels of CRP and ESR.…”
Section: Discussionmentioning
confidence: 99%
“…The MR imaging features of IMT of the thigh are nonspecific on T1 and T2 weighted images. The IMT of the thigh [ 13 ] is reported to preferably create a slow growing mass located at the medial aspect of the thigh. In our case, there was a slow-growing mass in the intermuscular interval between adductors and vastus medialis and intermedius.…”
Section: Discussionmentioning
confidence: 99%
“…Previous reports have shown that there is a moderate to intense enhancement of the lesion with the use of contrast substance; whereas in our case there was only mild enhancement best seen in delayed images, probably due to the presence of abundant fibrous tissue around the lesion. Han and Lin have also noted that the degree of enhancement is associated with the relative cellularity and the amount of fibrous tissue of the myofibroblastic tumor [ 13 , 36 ]. Delayed and mild enhancement after administration of gadolinium is usually seen in lesions with excessive fibrous component such as desmoids and nodular fasciitis [ 13 , 36 ].…”
Inflammatory myofibroblastic tumors are uncommon neoplasms; presentation of these tumors in the lower extremities is extremely rare. We present a case of a 47-year-old male with fever, fatigue, and a slow-growing thigh mass. The inflammatory markers were elevated and the MR images showed a well-defined intermuscular lesion with mild heterogeneous enhancement. The lesion was excised and histologic examination was consistent with an inflammatory myofibroblastic tumor. No adjuvant therapy was needed and the patient remained asymptomatic with no evidence of tumor recurrence during the 2 years of follow-up.
“…За длительный период существования данной «собирательной» нозологии были описаны наблюдения с вовлечением практически всех органов и тканей, чаще -с поражением ткани легкого и орбиты. В части наблюдений была установлена связь с местным воспалительным процессом, обусловленным предшествующей травмой (в т. ч. хирургической), аутоиммунным процессом, а также с опухолевым процессом по типу паранеопластической реакции [12][13][14][15][16][17][18][19][20][21][22].…”
The paper focuses on Epstein-Barr virus (EBV)-associated infl ammatory pseudotumor in the spleen, an extremely rare disease which so far remained undefi ned in the Russian literature. The morphology of it is not characterized by any specifi c features and is represented by a spindlecell component with pronounced infl ammatory infi ltration which complicates diff erential diagnosis. Previously this nosology was regarded within a group of the so-called infl ammatory pseudotumors due to the similarity of the clinical course and radiological presentation with tumor processes. Today the term "infl ammatory pseudotumor" is considered obsolete, since as pathogenesis was being studied within this disease group, the individual forms with similar morphology and diff erent histogenesis were identifi ed. Diff erential diagnosis in the context of additional diagnostic techniques is based in such cases on a wide range of reactive states, benign and malignant tumors.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.