Increased 68 Ga-FAPI Activity in Hepatic Inflammatory Myofibroblastoma.

ABSTRACT: Hepatic inflammatory myofibroblastoma is an uncommon borderline tumor. We present 68 Ga-FAPI PET/CT findings of hepatic inflammatory myofibroblastoma in a 58-year-old man. The rare hepatic inflammatory myofibroblastoma demonstrated intense tracer uptake on 68 Ga-FAPI PET/CT. This case demonstrates the potential value of 68 Ga-FAPI PET/CT for the evaluation of hepatic inflammatory myofibroblastoma.

Inflammatory myofibroblastic tumor of the kidney in patient with nephron-sparing surgery. Case report and review of the literature.

According to the WHO classification, mesenchymal tumors of the kidney are divided into mesenchymal tumors occurring mainly in adults and children. Accumulating evidence suggests that renal mesenchymal tumors represent a group of histologically heterogeneous diseases. We are reporting the case of a 58-year-old man with a history of nephron-sparing surgery due to renal cell carcinoma in 2014 in his left kidney. He was urgently admitted to the hospital due to left-sided nephrotoxic and anuria. Computed tomography (CT) imaging was performed and revealed irregular tumor mass. He underwent left-sided nephrectomy. Microscopic appearance and immunoprofile allowed to diagnose the tumor as an inflammatory myofibroblastic tumor (IMT) of the kidney. According to the 2016 World Health Organization (WHO) classification of tumors of the urinary system and male genital organs, IMT is a benign mesenchymal tumor with malignant potential. Typical locations are the liver and biliary tract, lung, and gastrointestinal tract. In the urogenital system, IMT occurs mainly in the bladder. So far, 48 cases have been reported in the kidney.

Mammary-type myofibroblastoma of the thigh mimicking liposarcoma.

Myofibroblastoma is a rare tumor that was originally described in the breast but has since been found in extra-mammary locations, where it been renamed mammary-type myofibroblastoma (MTM). We describe a case of MTM occurring in the anterior compartment of the thigh, which was initially interpreted as a possible a liposarcoma on MRI. Two subsequent biopsy samples and eventual excision revealed the tumor to have the characteristic pathology of MTM. This benign tumor, albeit rare, needs to be considered in the imaging differential diagnosis of fat containing deep soft tissue masses of the extremity.

A case of Crohn's disease combined with inflammatory myofibroblastoma of abdominal wall. / å ‹ç½—æ©ç— åˆå¹¶è ¹å£ç‚Žæ€§è‚Œçº¤ç»´æ¯ç»†èƒžç˜¤1例.

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.

Myofibroblastoma of the male breast: A case report / Miofibroblastoma en la mama de un varón, descripción de un caso clínico

Myofibroblastoma (MFB) is a rare spindle stromal tumour of the breast that predominates in 60–80 years-old adult males. Its imaging features are nonspecific, leading to misdiagnosis. Thus, core biopsy is needed for definitive diagnosis. Macroscopically, MFB is usually a well-circumscribed, firm and rubbery, unencapsulated, pale white to grey round mass. Microscopically, it consists on spindle cells arranged in haphazardly intersecting fascicles or clusters, thick hyalinized collagen bundles and low mitotic activity with a lack of myoepithelial component and necrosis. Immunohistochemistry shows consistently positive immunoreactivity to vimentin and CD34, while expression of desmin, SMA, bcl-2 and CD99 varies. Oestrogen, progesterone and androgen receptors are usually expressed. They are constantly negative to cytokeratins, EMA, S100 protein, HMB-45 and c-kit (CD117). These differentiate them from fibroadenoma, phyllodes tumour, round pattern gynecomastia, carcinoma and sarcoma, since they present infiltrative growth and are negative to CD34. Wide local excision is curative, with no need of sentinel lymph node biopsy, since local recurrence is extremely low and has been reported to be less than 1.5%. No distant metastases have been described on the literature. We report a rare case MFB on a 73-year-old male attended at our institution presenting with a nodule on the right breast. (AU)

El miofibroblastoma (MFB) es un tumour estromal de células fusiformes que aparece en varones de 60-80 años. Las características radiológicas son inespecíficas, por lo que es necesaria la realización de biopsia para el diagnóstico definitivo. Macroscópicamente se trata de una lesión bien circunscrita, firme, no encapsulada. Microscópicamente consiste en células fusiformes organizadas en fascículos entremezclados con bandas de colágeno hialino, con baja actividad mitótica y ausencia de componente mioepitelial y necrosis. La inmunohistoquímica muestra la expresión constante de vimentina y CD34, con expresión variable de desmina, AML, bcl-2 y CD99. Los receptores de estrógenos, progesterona y andrógenos normalmente son positivos, mientras que la expresión de citoqueratinas, EMA, S100, HMB-45 y c-kit (CD117) es negativa. Estas características lo diferencian del fibroadenoma, tumour filodes, ginecomastia, carcinoma y sarcoma, ya que la mayoría de ellos se caracterizan por ser negativos para CD34 y presentar crecimiento infiltrativo. La tumorectomía es considerada curativa, sin necesidad de realizar biopsia selectiva de ganglio centinela, dado que la recurrencia local es baja (menos del 1,5%). No se ha descrito la presencia de metástasis a distancia en la literatura. Presentamos el caso de MFB en un varón de 73 años que debutó con un nódulo en la mama derecha. (AU)

PET/CT-Detected myofibroblastoma of the breast with bizarre cells: A potential diagnostic pitfall of malignancy.

Myofibroblastoma (MFB) is a rare benign mesenchymal tumor usually occurring in the breast parenchyma. This tumor can present as a palpable nodule or can be incidentally detected as a nonpalpable mass on routine screening mammogram. We first report a rare case of histologically proven MFB of the breast revealed by fluoro-deoxyglucose uptake on PET-CT examination in a patient with a lung nodule. Tumor exhibited an unusual morphology, being predominantly composed of polygonal, epithelioid, and deciduoid-like cells set in a myxoid stroma. The most striking feature was the multifocal presence of atypical/bizarre, mono/bi-nucleated cells that, in addition to diffuse myxoid stromal changes, were a concern of malignancy, especially on core biopsy. The final diagnosis of MFB was achieved on surgically resected specimen and, similarly to other benign soft tissue tumors (especially leiomyoma and schwannoma/neurofibroma), the term "bizarre cell MFB of the breast" is proposed to emphasize the degenerative/reactive nature of the atypia.

Imaging Findings of Mammary Myofibroblastoma: A Rare Fat-Containing Entity.

ABSTRACT: Mammary myofibroblastoma (MFB) is a rare benign stromal neoplasm; its imaging features are rarely reported and nonspecific. We retrospectively analyzed a case of pathologically proved breast MFB that revealed indistinct high-density mass in mammography and hypoechoic ill-defined breast tumor in ultrasonography. Meanwhile, hypointense appearance on T2-weighted imaging of magnetic resonance imaging and containing fat tissue might be very valuable characteristics in the differential diagnosis of breast MFB.

Mammary and extramammary myofibroblastoma: multimodality imaging features with clinicopathologic correlation, management and outcomes in a series of 23 patients.

OBJECTIVE: The purpose of this study was to describe the imaging appearance, diagnosis, and management of mammary and extramammary myofibroblastoma (MFB) in a series of 23 patients. METHODS AND MATERIALS: Following institutional review board approval, cases were identified by searching for "myofibroblastoma" in radiology reports. Multimodality imaging and pathological features were assessed. RESULTS: 23 cases of myofibroblastoma were identified in 15 males and 8 females. Most cases were in the breast (20/23, 87%), presenting as a palpable mass or discovered incidentally on mammography in females or chest CT in males. Extramammary MFB lesions (3/23, 13%) presented with symptoms related to mass effect. At imaging, MFB most often demonstrated an oval or irregular mass that was hypoechoic or heterogeneously echogenic with variable margins. MRI showed T2 hyperintensity, diffusion restriction, and plateau kinetics. Extramammary MFB appeared as an enhancing mass with variable fat content and T2 intensity. CONCLUSION: Here we describe imaging and clinicopathological features of mammary and extramammary myofibroblastoma. ADVANCES IN KNOWLEDGE: Imaging description of this rare tumor is limited in the literature, and to date this is the largest case series describing the imaging findings.

Extramammary orbital myofibroblastoma: a rare orbital tumor.

Extra-mammary myofibroblastomas are rare benign mesenchymal neoplasms, histologically and immune-phenotypically identical to mammary myofibroblastomas. Histologically, they are characterized by the abundance of spindle cells in hyalinized collagenous stroma with positive expression of CD 34 and desmin. We present an extremely rare case of extra-mammary myofibroblastoma of the orbit in a 29-year - old male who presented with painless proptosis of the right eye, with a description of clinical, radiological, and histological findings.

Mammary-type Myofibroblastoma of the Pre-sacral Space: A Rare Neoplasm.

INTRODUCTION: Mammary-type myofibroblastoma is a very rare, benign, mesenchymal neoplasm that is histologically identical to a myofibroblastoma of the breast but located in an extra-mammary location. To our knowledge, there have been about 160 cases of extra-mammary myofibroblastoma reported to date. Our report describes a mammary-type myofibroblastoma located retro-rectally in the pre-sacral space. CASE REPORT: Our patient is a 55-year-old male that presented via referral for evaluation of a pelvic mass. He noted having a few loose stools since the mass had become apparent but did not report any other associated symptoms. An MRI of the pelvis revealed a 9 cm, fat containing, solid, retro-rectal mass within the pre-sacral space, which did not appear to be contiguous with the rectum, ureters, or pelvic sidewall. He elected to have the mass surgical removed. The mass was removed as a single specimen that measured 9.5 x 7.5 x 7.0 cm. By immunohistochemistry, the neoplastic cells show co-expression of desmin, CD34, estrogen receptor, and loss of RB1 expression, which is consistent with the diagnosis of mammary-type myofibroblastoma. DISCUSSION: Mammary-type myofibroblastoma is a very rare, benign, soft tissue neoplasm. These neoplasms most often present as a painless slow growing mass in a middle-aged male. Although exceedingly rare, mammary-type myofibroblastoma should be on the differential diagnosis of patients presenting with a mass that was found incidentally or one that is producing mass-effect symptoms. When found, these tumors should be investigated to rule out other more serious pathologies and removed due to their high curability with surgical resection.

Inflammatory myofibroblastic tumor arising from soft tissues of extremities harboring a novel CLIP2-ALK fusion.

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.