Spindle-shaped Tumor Cells Research Articles

Cutting the Gordian Knot: Rare Presentation of Nodular Fasciitis as Supraclavicular Swelling with Muscular Involvement in 68Ga-FAPI-PET/CT

Background: Nodular fasciitis is a benign, singularly occurring nodular fibroblastic/myofibroblastic neoplasia. Due to the rapid growth and cellular atypia, this rare differential diagnosis in the head and neck region can be mistaken for malignant sarcomas. Methods: We present a 40-year-old female patient with an unclear, rough, and poorly displaceable supraclavicular swelling on the right as part of a medical check-up. Sonographically, the lump was poorly circumscribed with little vascularization. A consecutive core needle biopsy of the lesion yielded inconclusive results showing spindle-shaped tumor cells. 68Ga-FAPI-PET/CT showed an intensive uptake of the right supraclavicular lesion in addition to postoperative changes in the right tonsil. Subsequent operative partial excision of the lesion confirmed the histopathological diagnosis of nodular fasciitis. Results: Nodular fasciitis is the most prevalent pseudosarcoma found in soft tissues. This case is the first description of 68Ga-FAPI-PET/CT in nodular fasciitis. Surgical removal is advised; nevertheless, the tumor frequently diminishes on its own, and recurrence is rare. Extensive surgical therapy is not necessary. Conlcusions: The recognition of nodular fasciitis and its benign characteristics is crucial to prevent diagnostic errors and the subsequent unnecessary operative treatment of the patient.

Clinico-radiological features of optic nerve sheath schwannoma: Review and illustrative case.

The optic nerve sheath is a rare site for schwannoma, to our knowledge 19 optic nerve sheath schwannoma (ONSS) cases have been reported. Difficulty can arise in diagnosis as imaging findings can be relatively non-specific. We describe a case of ONSS that mimicked orbital cavernous venous malformation pre-operatively. A 43-year-old woman presented with right subacute visual loss, reduced vision to 6/48 and signs of optic neuropathy. Endocapsular excision of the mass, which was adherent to the optic nerve, was accomplished from an endoscopic endonasal approach utilizing a 5-hand technique of retrocaruncular dissection. Histology showed spindle-shaped tumour cells with S100 positivity, consistent with ONSS. At 6 months post-operatively vision had improved to 6/6. We show that an endoscopic endonasal approach, augmented by transcaruncular retraction, can be utilized to excise ONSS with an excellent outcome. We review published cases of ONSS, including demographic and clinical features, differential diagnosis based on radiological features, and described outcomes, which are generally poor.

Solitary Myofibroma of the oral and maxillofacial regions in pediatric patients: A clinicopathological analysis of ten patients and review of 75 cases

BackgroundThe imaging manifestations of oral and maxillofacial myofibroma/myofibromatosis can vary among patients. Although many clinical cases have been reported, a consensus on the clinicopathological features of and treatment principles for this disease is lacking. PurposeThis study aimed to summarize the clinicopathological features of solitary myofibroma of the oral and maxillofacial regions in pediatric patients. MethodsThe clinical data, histological features, and immunohistochemical characteristics of ten pediatric patients who underwent surgical removal and subsequent pathological diagnosis of myofibroma were collected and retrospectively and cross-sectionally analyzed. ResultsSeven patients were male, and 3 were female, with ages ranging from 3 months to 6 years (mean: 2.6 years). The patients presented with solitary lesions involving the mandibular gingiva and adjacent mandible (4 patients), mandible (2 patients), oral floor and submandibular area and adjacent mandible (1 patient), gingiva (1 patient), maxilla (1 patient), and oropharynx (1 patient). Light microscopy revealed spindle-shaped tumor cells organized in bundles or vortex patterns, forming a hemangiopericytoma-like perivascular pattern, whereas immunohistochemical staining revealed diffuse smooth muscle actin (SMA) positivity. All patients underwent surgical resection, and none experienced recurrence over the 12- to 82-month follow-up. ConclusionsSolitary myofibroma in the oral and maxillofacial regions is predominantly observed in infants and young children, with a higher incidence among males. The prognosis is favorable following localized lesion resection or curettage of jawbone lesions. Accurate recognition of the clinical, radiological, and pathological features of the disease will reduce the misdiagnosis rate.

Primary pulmonary myxoid sarcoma in the interlobar fissure of the left lung lobe: a case report

BackgroundPrimary pulmonary myxoid sarcoma (PPMS) is a rare, low-grade malignant tumor, constituting approximately 0.2% of all lung tumors. Despite its rarity, PPMS possesses distinctive histological features and molecular alterations, notably the presence of EWSR1-CREB1 gene fusion. However, its precise tissue origin remains elusive, posing challenges in clinical diagnosis.Case demonstrationA 20-year-old male patient underwent a routine physical examination 6 months prior, revealing a pulmonary mass. Following surgical excision, microscopic evaluation unveiled predominantly short spindle-shaped tumor cells organized in a fascicular, beam-like, or reticular pattern. The stromal matrix exhibited abundant mucin, accompanied by lymphocytic and plasma cell infiltration, with Russell bodies evident in focal areas. Immunophenotypic profiling revealed positive expression of vimentin and epithelial membrane antigen in tumor cells, whereas smooth muscle actin and S-100, among others, were negative. Ki-67 proliferation index was approximately 5%. Subsequent second-generation sequencing identified the characteristic EWSR1-CREB1 gene fusion. The definitive pathological diagnosis established PPMS. The patient underwent no adjuvant chemotherapy or radiotherapy and remained recurrence-free during a 30-month follow-up period.ConclusionsWe report a rare case of PPMS located within the left lung lobe interlobar fissure, featuring Russell body formation within the tumor stroma, a novel finding in PPMS. Furthermore, the histomorphological characteristics of this case highlight the diagnostic challenge it poses, as it may mimic inflammatory myofibroblastic tumor, extraskeletal myxoid chondrosarcoma, or hemangiopericytoma-like fibrous histiocytoma. Therefore, accurate diagnosis necessitates an integrated approach involving morphological, immunohistochemical, and molecular analyses.

A Rare Tumor of Intravascular Myopericytoma in the Right Renal Vein.

Myopericytoma, a perivascular myoid neoplasm, is commonly found in the dermis or subcutaneous tissues; however, its occurrence in visceral organs is unusual. Here, we present an extremely rare tumor of intravascular myopericytoma of the right renal vein. A 44-year-old man was incidentally diagnosed with a mass in the right kidney during a routine checkup. A nephrectomy was performed because the urologist suspected renal cancer. A gross examination of the resected specimen revealed a well-circumscribed brown mass in the renal hilum. Histologically, the tumor showed a concentric multilayered proliferation of spindle cells surrounding blood vessels. Immunohistochemical staining showed that the spindle-shaped tumor cells were negative for desmin but positive for α-smooth muscle actin and h-caldesmon, indicating their myoid nature. We confirmed that the tumor was located in the right renal vein because it was encased within a thick wall that was desmin-positive and contained elastic fibers, as shown by Elastica van Gieson staining. The patient was diagnosed with an intravascular myopericytoma of the right renal vein. There are several differential diagnoses for renal mesenchymal tumors, including angiomyolipoma. This emphasizes the importance of considering these uncommon tumors when examining nephrectomy specimens.

A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa

BackgroundPEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration.Case presentationA 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa.ConclusionsA histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.

An Unusual Case of Scrotal Aggressive Angiomyxoma (AAM) Masquerading as Non-Reducible Inguinal Hernia – A Rare Case Report and Literature Review

This case report details a rare instance of scrotal Aggressive Angiomyxoma (AAM) in a 62-year-old male, presenting with a gradually enlarging mass masquerading as a non-reducible inguinal hernia. AAM, predominantly found in adult females, is an exceptionally uncommon occurrence in men. The diagnostic challenge lay in the rarity of male cases and the tumor’s mimicry of common conditions like inguinal hernia. Upon physical examination, a non-reducible, non-tender swelling extending from the right inguinal region to the scrotal sac was noted. Radiological investigations initially suggested a scrotal mass or non-reducible inguinal hernia. Intraoperative exploration revealed a large encapsulated cystic mass, measuring 12 x 8 cm, originating from the inguinal ligament. Histopathological examination confirmed the diagnosis of aggressive angiomyxoma, characterized by spindle-shaped tumor cells, thick-walled blood vessels, and myxoid texture. The discussion underscores the diagnostic complexities of AAM, emphasizing the necessity of histopathological examination for conclusive diagnosis. A review of male AAM cases in the literature highlights similarities with female cases, supporting the expression of estrogen (ER) and progesterone receptors (PR). Surgical removal with clear margins is the primary treatment, although recurrence rates vary. The potential role of hormonal therapy, especially in ER and PR positive cases, is noted but requires further exploration. In conclusion, this rare case sheds light on the clinical and pathological characteristics of scrotal AAM in men. The diagnostic journey underscores the importance of considering AAM in the differential diagnosis of scrotal masses. Long-term follow-up is crucial due to the tumor’s propensity for local recurrence, making this case a valuable addition to the limited literature on male AAM.

Jejunal gastrointestinal stromal tumor that developed in a patient with neurofibromatosis type 1: a case report

BackgroundNeurofibromatosis type 1 (NF1) is known to be associated with the frequent occurrence of unique gastrointestinal stromal tumors (GISTs), preferably occurring in the small intestine, with no mutations in the c-kit proto-oncogene or platelet-derived growth factor receptor-alpha (PDGFRA), with a high tendency for multifocal development, indolent nature, with low proliferation activity and favorable prognosis.Case presentationA woman in her forties visited her local doctor complaining of menstrual pain; a large mass was detected in her lower abdomen, and she was referred to our hospital. The patient had hundreds of skin warts and café au lait spots. The patient’s mother had been diagnosed with type 1 neurofibromatosis. The patient met the diagnostic criteria for NF1 and was diagnosed with NF1. Ultrasonography showed a large heterogeneous cystic mass with various echo patterns, solid compartments and multiple septations. Magnetic resonance imaging showed a multilocular cystic mass with liquid content exhibiting various intensities, including that of blood. A small round solid mass was also observed close to the cystic tumor. Contrast-enhanced computed tomography showed that the round solid mass showed strong enhancement in the early phase, unlike the cystic tumor component. Open laparotomy revealed a multicystic exophytic tumor measuring 11.5 cm originating from the jejunal wall, 20 cm distal to the duodenojejunal flexure. A solid tumor measuring 2.1 cm was also found on the anal side of the large tumor. We resected the short segment of the jejunum, including the two lesions. Microscopic findings revealed that the cystic and solid tumors consisted of spindle-shaped tumor cells showing little atypia with a fascicular or bundle arrangement. Nuclear mitosis was scarce. Immunostaining of the tumor cells showed positive staining for KIT and DOG1 and negative staining for S100 and desmin. The NF1 patient was diagnosed with multiple GISTs accompanied by intratumoral hemorrhagic denaturation arising from the jejunum. The TNM staging was pT4N0M0, stage IIIA.ConclusionWe report a case of GISTs associated with NF1 that showed a jejunal origin, multifocal development and few mitotic figures. The recurrence risk, survival prognosis and need for adjuvant chemotherapy, particularly in cases where the initial GIST exhibits a very indolent pathology in NF1-related GISTs, remain to be elucidated.

Dermatofibrosarcoma Protuberans; Case Report in a Pediatric Patient

Dermatofibrosarcoma protuberans (DFSP) is a rare and locally aggressive mesenchymal neoplasm arising from the dermal and subcutaneous tissues. It typically manifests as a slow-growing, firm, protuberant or plaque-like lesion on the skin. DFSP predominantly affects young to middle-aged adults, with a slight predilection for females. This tumor exhibits a distinctive infiltrative growth pattern, characterized by tumor cells invading the surrounding tissues, including the deep subcutaneous layers and the underlying fascia. Histopathologically, DFSP presents characteristic features, such as the presence of spindle-shaped tumor cells arranged in a "storiform" pattern, as well as the presence of a prominent "honeycomb" appearance due to the presence of multinucleated giant cells. Immunohistochemical staining is essential to confirm the diagnosis, with positivity for CD34 being a hallmark of DFSP. Although DFSP rarely metastasizes, its extensive local invasion and high recurrence rate necessitate a wide surgical excision with clear margins as the mainstay of treatment. The use of Mohs micrographic surgery or adjuvant radiation therapy has demonstrated favorable outcomes in cases with challenging anatomical locations or microscopically positive margins. Imatinib mesylate, a tyrosine kinase inhibitor, has also emerged as a promising systemic therapy for advanced or unresectable DFSP, particularly cases with the COL1A1-PDGFB gene fusion, which represents the molecular hallmark of DFSP. Early diagnosis and appropriate management are crucial to achieving optimal outcomes in DFSP. This article provides a comprehensive review of the clinical, histopathological, and molecular aspects of DFSP, highlighting the significance of a multidisciplinary approach to ensure accurate diagnosis and tailored therapeutic strategies for patients affected by this rare dermatological neoplasm.

Unusual presentation of Giant Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, soft tissue sarcoma with a low metastatic potential but high rate of recurrence after surgical intervention. This tumor is characterized by a long history of slow growth. DFSP usually occurs on trunk and proximal extremities but rarely touches distal extremities such as hands, fingers, or foot below knees. We present a case of giant DFSP of the distal extremity, with a post-traumatic onset in childhood and a very long course. A 45-year-old patient presented with a tumoral lesion of the foot, evolving since 35 years with an onset at the age of 10 years, after a strong trauma of the 3rd toe. Physical examination revealed a reddish purple polynodular neoplastic lesion with a firm consistency. One of these nodular masses was ulcerated with areas of necrosis. The CT scan did not detect any bone involvement. Magnetic resonance imaging revealed a heterogeneously enhancing mass lesion on the dorsal aspect of the left forefoot. In depth, this mass invaded the dorsal interosseous muscles. The histopathological study was in favor of DFSP, with evidence of spindle-shaped tumor cells arranged in a storiform pattern. On immunohistochemistry (IHC), the cells expressed intense and diffuse CD34 and were negative for Desmin and S-100. The localization or occurrence of DFSP after a long period of trauma should not mislead the diagnosis. A careful histological study with IHC should be carried out in order to set up an appropriate treatment.

Biphenotypic Sinonasal Sarcoma: A Genetically Confirmed Case Showing Bone Invasion Accompanying a Non-neoplastic Respiratory Epithelium.

Biphenotypic sinonasal sarcoma is a newly established tumor entity that is associated with distinct clinicopathological findings. Biphenotypic sinonasal sarcoma is a rare, low-grade spindle cell sarcoma that arises in middle-aged females, exclusively in the sinonasal tract. A fusion gene involving PAX3 is detected in most biphenotypic sinonasal sarcomas, which aids in its diagnosis. Here, we report a case of biphenotypic sinonasal sarcoma with its cytological findings. The patient was a 73-year-old woman who presented with purulent nasal discharge and dull pain in the left cheek area. Computed tomography showed a mass extending from the left nasal cavity to the left ethmoid sinus, the left frontal sinus, and the frontal skull base. She underwent a combined transcranial and endoscopic approach for en bloc resection with a safety margin. Histologically, spindle-shaped tumor cells have been thought to proliferate mainly in the subepithelial stroma. Here, nasal mucosal epithelial hyperplasia was noted, and the tumor had invaded the bone tissue accompanying the epithelial cells. Fluorescence in situ hybridization (FISH) analysis showed a PAX3 rearrangement, and next-generation sequencing identified a PAX3::MAML3 fusion. Based on FISH, split signals were observed not in respiratory cells but in stromal cells. This indicated that respiratory cells were non-neoplastic. In the diagnosis of biphenotypic sinonasal sarcoma, the inverted growth of the respiratory epithelium can be a diagnostic pitfall. FISH analysis using a PAX3 break-apart probe is helpful not only for an accurate diagnosis but also for detecting the true neoplastic cells.

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Digestive EndoscopyVolume 35, Issue 2 p. i-i Cover ImageFree Access Cover Image First published: 19 January 2023 https://doi.org/10.1111/den.14358AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Graphical Abstract Cover image: Endoscopic full-thickness resection of a gastric gastrointestinal stromal tumor (GIST). (a) A 30 mm, intraluminal type submucosal tumor at the greater curvature of the upper body is diagnosed as GIST by boring biopsy. (b) After mucosal incision and slight submucosal dissection, a clip with line is attached to the specimen for easy dissection and for preventing the tumor from extending beyond the stomach. (c) En bloc resection is achieved, and abdominal fat is identified from the perforation site. (d) The defect is closed with a purse-string suture. (e) The defect is closed completely. (f) The tumor is retrieved piecemeal. (g) Sectioning after fixation of all the specimens. (h) Spindle-shaped tumor cells proliferate in an intricate fascicle, with positive c-kit, leading to the diagnosis of GIST. (See Shichijo et al. p. 209). Volume35, Issue2Special Issue: Transluminal Endoscopy and Endoscopic Full-thickness ResectionJanuary 2023Pages i-i RelatedInformation

Primary fibrosarcoma of the distal femur. Report of a rare case

Fibrosarcoma of bone is a relatively rare malignant neoplasia, representing less than 5% of all bone tumors. It is distinguished from other bone sarcomas by the presence of spindle-shaped tumor cells located among intertwining bundles of collagen fibers, without any other type of histological differentiation, such as the formation of cartilage or bone.
 In the present report, we present a rare case of primary fibrosarcoma in the distal femur in a 45-year-old patient with complaints of mild pain and swelling in the right knee joint for approximately 6 months. After imaging evaluation, a biopsy was performed; in the second stage, a subsequent resection of 16 cm of the distal femur and its replacement with a tumor endoprosthesis was performed. Three months postoperatively, no local recurrence or metastasis of the tumor was observed.

A case of ALK-positive histiocytosis with multiple lesions in the unilateral breast: A case report.

Introduction and importancePrimary ALK-positive histiocytosis of the breast is rare. Here, we report a case of ALK-positive histiocytosis with multiple unilateral breast lesions.Case presentationOur patient was a 38-year-old female with primary ALK-positive histiocytosis of the breast with multiple lesions. There were no lesions in other organs, and the patient was considered surgically resectable and underwent a left total mastectomy and sentinel lymph node biopsy. Histopathologically, there were at least three lesions in the left breast in upper inner quadrant (UIQ), upper quadrant (UQ), and upper outer quadrant (UOQ). All lesions showed spindle-shaped tumor cells that were positive for CD163 and ALK and negative for AE1/AE3. Fluorescence in situ hybridization (FISH) showed ALK and KIF5B rearrangements, suggesting the presence of the KIF5B-ALK fusion gene. In conclusion, this case was confirmed to be ALK-positive histiocytosis with multiple lesions in the unilateral breast. The patient underwent surgery and was discharged without complications.Clinical discussionReports of ALK-positive histiocytosis are very rare, and reports of primary cases in the breast are even rarer. The basic treatment for ALK-positive histiocytosis is surgical resection; however, ALK inhibitors may be effective in unresectable or disseminated cases. Accurate diagnosis at the time of initial treatment is necessary to expand the treatment options.ConclusionThis is the first case of ALK-positive histiocytosis with multiple lesions in the unilateral breast.

ALK rearrangement-associated renal cell carcinoma morphologically mimicking mucinous tubular and spindle cell carcinoma: a case report

BackgroundAnaplastic lymphoma kinase rearrangement-associated renal cell carcinoma (ALK-RCC) is an extremely rare tumor and ALK-RCC that mimics mucinous tubular and spindle cell carcinoma (MTSCC) has been very reported only in one instance.Case presentationA 42-year-old Japanese woman was admitted to our hospital for the treatment of a left renal tumor measuring 5 cm in maximum dimension. She underwent a laparoscopic left nephrectomy. Histologically, the tumor formed tubular or focally papillary structures with a small amount of spindle-shaped tumor cells against the background of prominent extracellular mucin. Although the tumor cells were negative for immunohistochemistry (IHC) of alpha-methylacyl-CoA racemase (AMACR) and lymph node metastasis was presented (these are atypical findings for MTSCC), we initially diagnosed the tumor as MTSCC based on its morphological characteristics with mucin deposition. However, an additional IHC analysis revealed that the tumor cells were diffusely positive for ALK-IHC. In addition, TPM3 exon 8 – ALK exon 20 fusion gene was detected by RNA sequencing. The tumor was thus correctly diagnosed as ALK rearrangement-associated renal cell carcinoma (ALK-RCC).ConclusionsSince the use of molecular targeted therapy with an ALK inhibitor for ALK-RCC is promising, the correct pathological diagnosis of ALK-RCC is quite important. We strongly recommend that ALK-IHC be routinely performed for renal tumors with negative AMACR staining that mimic MTSCC.

Impact of Neoadjuvant Bevacizumab on Neuroradiographic Response and Histological Findings Related to Tumor Stemness and the Hypoxic Tumor Microenvironment in Glioblastoma: Paired Comparison Between Newly Diagnosed and Recurrent Glioblastomas.

BackgroundPreviously, we reported that bevacizumab (Bev) produces histological and neuroradiographic alterations including changes in tumor oxygenation, induction of an immunosupportive tumor microenvironment, and inhibition of stemness. To confirm how those effects vary during Bev therapy, paired samples from the same patients with newly diagnosed glioblastoma (GBM) who received preoperative neoadjuvant Bev (neoBev) were investigated with immunohistochemistry before and after recurrence.MethodsEighteen samples from nine patients with newly diagnosed GBM who received preoperative neoBev followed by surgery and chemoradiotherapy and then autopsy or salvage surgery after recurrence were investigated. The expression of carbonic anhydrase 9 (CA9), hypoxia-inducible factor-1 alpha (HIF-1α), nestin, and Forkhead box M1 (FOXM1) was evaluated with immunohistochemistry.For comparison between neoBev and recurrent tumors, we divided the present cohort into two groups based on neuroradiographic response: good and poor responders (GR and PR, respectively) to Bev were defined by the tumor regression rate on T1-weighted images with gadolinium enhancement (T1Gd) and fluid-attenuated inversion recovery images. Patterns of recurrence after Bev therapy were classified as cT1 flare-up and T2-diffuse/T2-circumscribed. Furthermore, we explored the possibility of utilizing FOXM1 as a biomarker of survival in this cohort.ResultsA characteristic “pseudo-papillary”-like structure containing round-shaped tumor cells clustered adjacent to blood vessels surrounded by spindle-shaped tumor cells was seen only in recurrent tumors. Tumor cells at the outer part of the “pseudo-papillary” structure were CA9-positive (CA9+)/HIF-1α+, whereas cells at the inner part of this structure were CA9−/HIF-1α+ and nestin+/FOXM1+. CA9 and HIF-1α expression was lower in T1Gd-GR and decreased in the “T2-circumscribed/T2-diffuse” pattern compared with the “T1 flare-up” pattern, suggesting that tumor oxygenation was frequently observed in T1Gd-GR in initial tumors and in the “T2-circumscribed/T2-diffuse” pattern in recurrent tumors. FOXM1 low-expression tumors tended to have a better prognosis than that of FOXM1 high-expression tumors.ConclusionA “pseudo-papillary” structure was seen in recurrent GBM after anti-vascular endothelial growth factor therapy. Bev may contribute to tumor oxygenation, leading to inhibition of stemness and correlation with a neuroimaging response during Bev therapy. FOXM1 may play a role as a biomarker of survival during Bev therapy.

Dermal lipofibromatosis-like neural tumor.

Lipofibromatosis-like neural tumor (LPF-NT) is a rare soft tissue typically occurring in the subcutis, characterized by a cellular proliferation of CD34- and S100-protein positive spindle-shaped tumor cells with an infiltrative growth pattern. To describe five cases arising mainly in the dermis in order to expand their morphologic spectrum. H&E slides were reviewed, and all cases were stained for CD34, SOX10, S100, ALK, and NTRK1 and some of them with additional staining. Patients were three males and two females with a mean age of 44.8 years (14-68 years). Histopathologically, all cases were characterized by a dense dermal infiltration by monomorphous, mildly atypical, plump to spindle-shaped tumor cells, staining diffusely positive for CD34, S100, and NTRK1 but were negative for S100, EMA, NKIC3, MNF116, SMA, ALK, and desmin. Limited clinical information. LPL-NT can be located mainly in the dermis. Sixty percent of our cases showed typical areas of LPL-NT intermingled with more plump cells like the ones in fibrous hamartoma of infancy. We recommend a panel of CD34, S100, and NTRK1 antibodies not only in subcutaneous spindle cell neoplasms but also in the ones predominantly involving the dermis in order to make an accurate diagnosis.

Epithelioid angiosarcoma of the duodenum: a case report

BackgroundAngiosarcomas are rare malignant tumors that arise from the endothelium of blood vessels. They occur most commonly in the skin and soft tissue, and less commonly in the breast, liver, bone, and spleen. Gastrointestinal angiosarcomas are extremely rare. Herein, we present a case of duodenal epithelioid angiosarcoma that was treated with surgical resection.Case presentationA 68-year-old man presented with a 1-month history of fatigue and hypotension. He visited the outpatient clinic for a routine follow-up. Laboratory examination revealed anemia. Esophagogastroduodenoscopy revealed multiple duodenal lesions with central ulceration. A biopsy showed a sheet-like arrangement of large round and spindle-shaped tumor cells that were positive for CD31. Based on the histological and immunohistochemical staining findings, an epithelioid angiosarcoma was diagnosed. Computed tomography (CT) and positron emission tomography–CT revealed no lymph node metastasis or distant metastasis. Radical subtotal stomach-preserving pancreatoduodenectomy with lymphadenectomy was performed. After removing the specimen, reconstruction was performed using the Child procedure. Grossly, two dark-red polypoid tumors were found in the second portion of the duodenum. Histological evaluation revealed proliferation of malignant round and polygonal cells arranged in sheets and spindle-like cells arranged in bundles. Vasoformative structures were recognized as slit-like spaces containing red blood cells. Immunohistochemical staining demonstrated that the tumor cells were positive for CD31. These findings confirmed the diagnosis of epithelioid angiosarcoma in the duodenum. The patient’s postoperative course was uneventful. The patient was discharged on postoperative day 19 without any complications. At a follow-up examination in the outpatient clinic at postoperative 4 months, no evidence of recurrence was detected.ConclusionThe present report describes a case of duodenal epithelioid angiosarcoma. Duodenal angiosarcomas may cause anemia and gastrointestinal bleeding. Because angiosarcomas sometimes show epithelioid cytomorphology, immunohistochemical analysis is useful for confirming the diagnosis.

Myxoid type and non-myxoid type of intimal sarcoma in large vessels and heart: review of histological and genetic profiles of 20 cases.

Intimal sarcoma is one of the most common and well-known primary malignant neoplasms of the aorta and heart. The authors reviewed cases of intimal sarcoma from histological, immunohistochemical and genetic perspectives. Twenty cases of intimal sarcoma were retrieved. Immunohistochemistry and FISH of MDM2 and PDGFRA genes were performed. All 20 tumours were composed of spindle-shaped, stellate, oval or polygonal tumour cells with irregular hyperchromatic nuclei arranged in a haphazard pattern, accompanied by nuclear pleomorphism and frequent mitotic figures. Other histological findings were as follows: abnormal mitosis in 10 cases (50%), necrosis in 15 cases (75%), myxoid stroma in 12 cases (60%), cartilaginous formation in 1 case (5%), haemorrhage in 12 cases (60%) and fibrinous deposition in 14 cases (70%). The tumours were positive for MDM2 in 16 cases (80%), ERG in 4 cases (20%), alpha-smooth muscle actin in 6 cases (30%), desmin in 5 cases (25%) and AE1/AE3 in 4 cases (20%). Immunohistochemical positivity was focal in each case. Loss of H3K27me3 expression was noted in 2 cases (10%). MDM2 and PDGFRA gene amplifications were detected in 11 cases (55%) and 1 case (5%), respectively. Fisher's exact test revealed a significant correlation between MDM2 gene amplification and myxoid stroma (p = 0.0194). No parameters showed any association with the anatomical location of the tumours. It was suggested that myxoid histology of intimal sarcoma may be associated with MDM2 gene amplification and that intimal sarcoma may be divided into myxoid and non-myxoid types.

S1-guideline atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS).

Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous neoplasms representing histomorphological, genetic as well as epigenetic variants of a disease spectrum. Both tumors typically manifest as nonspecific, often ulcerated, skin- to flesh-colored nodules in chronically sun-damaged skin of elderly male patients. AFX is a rather well demarcated, often rapidly growing tumor. PDS tumors are poorly circumscribed and are characterized by aggressive infiltrative growth. Fast as well as slow growth behavior has been reported for both tumors. Histologically, both are composed of spindle-shaped and epithelioid tumor cells with pleomorphic nuclei as well as atypical multinucleated giant cells. Atypical mitoses are common. In contrast to AFX, PDS involves relevant parts of the subcutis and shows areas of tumor necrosis and/or perineural infiltration. Due to the poorly differentiated nature of AFX/PDS (Grade3), histopathologically similar cutaneous sarcomas, undifferentiated carcinomas, melanomas and other diseases have to be excluded by immunohistochemical analysis. The treatment of choice is micrographically controlled surgery. In cases of AFX, a cure can be assumed after complete excision. Local recurrence rates are low as long as PDS tumors are surgically removed with a safety margin of 2cm. Metastasis is rare and mostly associated with very thick or incompletely excised tumors; it mainly affects the skin and lymph nodes. Distant metastasis is even more rare. No approved and effective systemic therapy has been established.