[Intraosseous cavernous hemangioma of the middle turbinate. Clinical cases]. / Vnutrikostnaya kavernoznaya gemangioma srednei nosovoi rakoviny. Klinicheskie sluchai.

Intraosseous vascular pathology of the turbinates is extremely rare in the practice of an otorhinolaryngologist and can be presented in various histopathological variants. The article presents two clinical cases in which an intraosseous cavernous hemangioma was hidden under the mask of a hypertrophied middle turbinate. The final diagnosis was established by the results of histological examination. The analysis of these clinical cases indicates that, despite the low prevalence, atypical clinical and CT picture, intraosseous formations of the nasal cavity can be of a vascular nature and certainly require a comprehensive examination, including CT, CT with contrast and/or MRI of the nose and paranasal sinuses. These clinical observations indicate that preliminary embolization of feeding vessels before surgical treatment is not required.

Complete and partial replacement of the inferior vena cava with autologous peritoneum in cancer surgery.

Resection of the inferior vena cava may be required in the courses of oncological surgeries for the tumors originating from or invading it. Management of the remaining defect depends on the extension of the resection. Partial or complete replacement of the inferior vena cava, with a patch or interposition graft, may be required. Standard techniques for the reconstruction with a prosthetic material or the autologous veins can be associated with the prosthetic graft infection, high cost, long-standing anticoagulation, technical difficulties, and/or need for extra incisions. The use of the autologous peritoneum represents an easy and inexpensive alternative for the partial and complete inferior vena cava reconstructions.

Cutaneous symplastic hemangioma: A series of four cases.

Symplastic hemangiomas (SH) are benign vascular lesions that show atypia in vascular smooth muscle and interstitial cells with sparing of endothelial cells. We present four cases of this rare tumor. The patients (two males; two females) ranged in age from 57 to 83 years (median 74); lesions were located on the leg (n = 3) and back (n = 1), and ranged from 6 to 8 mm. SH were well-circumscribed and dermal-based, often with an epidermal collarette (3/4). They were characterized by the presence of variably atypical, hyperchromatic/pleomorphic epithelioid to spindled cells within vascular walls (3/4) and/or perivascular stroma (4/4). Atypical multinucleated cells were present in three of four cases. The overall mitotic rate was low (0-2 mitotic figures per 10 HPFs; mean 0.75 per 10 HPFs), but atypical mitotic figures were seen in two of four cases. Atypical cells were negative for SMA (0/2), desmin (0/2), AE1/3 (0/2), and CAM5.2 (0/1). ERG, CD31, and CD34 stains were positive in endothelial cells but negative in atypical cells (4/4). Lesional cells and vessels were negative for podoplanin (3/3). Symplastic hemangioma is a benign tumor with bizarre atypia that may be mistaken for malignancy. We present four cases of this rare entity to increase awareness of this tumor and discuss the differential diagnosis.

Vascular neoplasms and non-neoplastic vascular lesions of the spleen.

Vascular neoplasms are among the most common conditions affecting the spleen. The majority of these are idiopathic, benign in nature and asymptomatic and therefore treated with a conservative management. Only rare cases cause splenomegaly and/or chronic consumption coagulopathies, thus requiring splenectomy. Among these, the most common is splenic hemangioma, followed by littoral cell angioma and lymphangioma. Peliosis is a peculiar tumor-like non-neoplastic vascular lesion that diffusely affects the spleen and frequently presents with concomitant hepatic involvement. As a distinctive feature, peliosis can occur as a secondary manifestation of infections, malignancies and in individuals using certain drugs. On the opposite spectrum of clinical behavior lies splenic angiosarcoma, a vascular endothelial malignancy with aggressive presentation and poor prognosis. In some cases the endothelial nature of this neoplasm may not be evident on routine histologic examination and immunohistochemistry is used to disclose such phenotype. The term hemangioendothelioma is rarely used to describe borderline vascular neoplasms which appear more aggressive than conventional hemangiomas, but that do not entirely fulfill the diagnostic criteria for angiosarcoma. Some of these neoplasms coexpress endothelial and histiocytic markers and therefore have been proposed as the borderline counterpart of littoral cell angioma. The existence of hemangioendothelioma as a diagnostic entity per se is debated and this diagnosis should be rendered with caution. The current review aims at highlighting the main histologic features of vascular neoplasms and non-neoplastic vascular lesions of the spleen.

A case of lymphangioma circumscriptum with new reflectance confocal microscopy findings.

In vivo reflectance confocal microscopy (RCM) findings of lymphangiomas have been scarcely reported. We report a lymphangioma circumscriptum (LC) with some new observations.

Modulation of LIN28B/Let-7 Signaling by Propranolol Contributes to Infantile Hemangioma Involution.

OBJECTIVE: Infantile hemangiomas (IHs) are the most common benign vascular neoplasms of infancy, characterized by a rapid growth phase followed by a spontaneous involution, or triggered by propranolol treatment by poorly understood mechanisms. LIN28/let-7 axis plays a central role in the regulation of stem cell self-renewal and tumorigenesis. However, the role of LIN28B/let-7 signaling in IH pathogenesis has not yet been elucidated. APPROACH AND RESULTS: LIN28B is highly expressed in proliferative IH and is less expressed in involuted and in propranolol-treated IH samples as measured by immunofluorescence staining and quantitative RT-PCR. Small RNA sequencing analysis of IH samples revealed a decrease in microRNAs that target LIN28B, including let-7, and an increase in microRNAs in the mir-498(46) cistron. Overexpression of LIN28B in HEK293 cells induced the expression of miR-516b in the mir-498(46) cistron. Propranolol treatment of induced pluripotent stem cells, which express mir-498(46) endogenously, reduced the expression of both LIN28B and mir-498(46) and increased the expression of let-7. Furthermore, propranolol treatment reduced the proliferation of induced pluripotent stem cells and induced epithelial-mesenchymal transition. CONCLUSIONS: This work uncovers the role of the LIN28B/let-7 switch in IH pathogenesis and provides a novel mechanism by which propranolol induces IH involution. Furthermore, it provides therapeutic implications for cancers in which the LIN28/let-7 pathway is imbalanced.

[Diagnosis and management of vascular malformations : Interdisciplinary teamwork in demand]. / Diagnostik und Management von Gefäßmalformationen : Interdisziplinäres Teamwork gefragt.

Vascular anomalies are very rare, but can occur in children and adults in almost every region of the body. Due to the complexity of this disease, the path to a definitive diagnosis is often difficult. It requires interdisciplinary teamwork with close exchange of information between the individual treatment partners to reach the correct diagnosis and then to start the best therapy. This article provides an overview of the main types of vascular malformations from a clinical, imaging, and histological point of view, following the current classification of the International Society for the Study of Vascular Anomalies (ISSVA).

Diagnosis and management of rare inferior vena cava leiomyosarcoma guided by a novel minimally invasive vascular biopsy technique.

Primary vascular tumors such as vascular leiomyosarcomas are rare, but exhibit markedly different characteristics than tumors that invade the vasculature from a secondary source. Establishing a diagnosis is essential in determining the appropriate treatment plan, but obtaining a histologic specimen may prove challenging and carry significant risks. Minimally invasive endovascular biopsy techniques can be pivotal in the diagnosis-and thus in the management-of vascular tumors. We present a case of a primary inferior vena cava leiomyosarcoma, not able to be adequately assessed by noninvasive imaging and deemed too risky to be approached with traditional percutaneous biopsy techniques. Accurate diagnosis of such tumors is critical, as the success of surgical resection, although high risk, depends greatly upon the type, location, and extent of malignancy.

[Study on the relationship between laparoscopic operation and pseudo vascular invasion in stage â a endometrial carcinoma].

Objective: To explore relations between pseudo vascular invasion (PVI) and laparoscopic operation in patients with stage Ⅰa endometrial carcinoma. Methods: Details of stage Ⅰa endometrial carcinoma patients' clinical information and pathological sections who received operation treatment between January 2008 and October 2015 were obtained from the Dalian Maternal and Child Health Hospital. There were 515 cases entrolled into this study, including 332 (64.5%) cases performed surgery by laparoscopy and 183 (35.5%) cases undergone abdominal surgery. The vascular invasion rate and the microscopic manifestations of PVI in two groups were retrospectively compared and analyzed. The follow-up period ended February 2017, the adjuvant treatment and prognosis of patients with lympho-vascular space invasion (LVSI) and PVI were analysed. Results: (1) Seventy-five of 515 cases with stage Ⅰa endometrial carcinoma, there were seven-five cases found with tumor cells in lympho-vascular space, including 52 cases of LVSI and 25 cases of PVI (2 cases of both). The incidence of cancer emboli in lympho-vascular space and LVSI in laparoscopic group and laparotomy group were respectively 15.4% (51/332) vs 13.1% (24/183) and 9.6% (32/332) vs 10.9% (20/183), there were no significant differences (all P>0.05). But the rate of PVI between the two group was significantly different [6.3% (21/332) vs 2.2% (4/183), P=0.036], laparoscopic group was higher than that in laparotomy group. (2) Of 332 cases in laparoscopic group, 21 cases was found PVI, containing 8 cases showed tumor tissue with mesenchyme or papillary structures located in large thick wall vessels of the outer lateral myometrium, 13 cases showed the tumor tissue was away from the vessel wall. Among 183 cases in laparotomy group, 3 cases with PVI which the tumor tissue was away from the vessel wall, and 1 with interstitial tissue. (3) During the follow-up period, 7 of 75 cases (both LVSI and PVI) were lost, and 66 cases were relapse-free survival, 2 cases recurred at fifth months and died respectively at 7, 20 months after the operation. This 2 cases were both PVI of laparoscopic group, and the pathological type was endometrioid adenocarcinoma grade 3, and adjuvant treatment was not accepted after the operation. The relapse-free survival with LVSI was 49 months and 46 months for cases with PVI. Conclusion: s Laparoscopic operation could increase PVI in patients with stage Ⅰa endometrial carcinoma, while does not increase LVSI. It is not yet possible to demonstrate that PVI affects the prognosis of patients.

[Characteristics of ERG, Fli-1, CD34, CD31 and Fâ §RAg expression in hepatic malignant vascular tumors].

Objective: To investigate the expression of ERG, Fli-1, CD34, CD31 and factor Ⅷ-related antigen(FⅧRAg) in hepatic malignant vascular tumors. Methods: A retrospective analysis was conducted on 63 cases of primary hepatic malignant vascular tumors and 31 cases of hepatic other malignant spindle cell tumors collected during January 1986 to January 2014. EnVision method was used to detect the expression of ERG, Fli-1, CD34, CD31, FⅧRAg. Results: Sixty-three cases of malignant vascular tumors, including 24 cases of angiosarcoma, 38 cases of epithelioid hemangioendothelioma and 1 case of hepatic Kaposi's sarcoma. All of the cases were positive for ERG(100.0%, 63/63). Positive rate of Fli-1, CD34, CD31, FⅧRAg was 96.8% (61/63), 87.3% (55/63), 81.0% (51/63) and 41.3% (26/63), respectively. In other hepatic malignant spindle cell tumors, the positive rate of ERG, Fli-1, CD34, CD31 and FⅧRAg was 3.2% (1/31), 19.4% (6/31), 19.4% (6/31), 9.7%(3/31) and 3.2%(1/31), respectively.The sensitivity of ERG, Fli-1, CD34, CD31, FⅧRAg was 100.0%, 96.8%, 87.3%, 81.0% and 41.3%, respectively.The specificity was 96.8%, 80.6%, 80.6%, 90.3% and 96.8%, respectively. Conclusion: ERG is a more sensitive and specific diagnostic marker for hepatic malignant vascular tumors in comparison to Fli-1, CD34, CD31 and FⅧRAg.

Local treatment with electrochemotherapy of superficial angiosarcomas: Efficacy and safety results from a multi-institutional retrospective study.

BACKGROUND: Angiosarcoma is an aggressive vascular neoplasm with a high propensity for local recurrence. Electrochemotherapy is an emerging skin-directed therapy, exerting prominent cytotoxic activity, and antivascular effects. Its efficacy in angiosarcoma has not been investigated. METHODS: This multicenter retrospective analysis reviewed patients who underwent electrochemotherapy from 2007 to 2014 for superficial advanced angiosarcomas. Bleomycin was administered intravenously and delivered within tumors by means of percutaneously applied electric pulses, according to the European Standard Operating Procedures for Electrochemotherapy. Tumor assessment was performed using RECIST (version 1.1). Toxicity (CTCAE, v4.0) and local progression-free survival (LPFS) were also evaluated. RESULTS: Nineteen patients (13 with locally advanced and 6 with metastatic angiosarcomas) were treated. Tumor sites were: scalp (n = 5), breast (n = 8), other skin sites (n = 3), and soft tissue (n = 3). Target lesions (n = 54) ranged in size from 1.5 to 2.5 cm (median, 2 cm). Treatment was well tolerated. After 2 months, an objective response was observed in 12/19 (63%) patients, complete in 8 (42%). One-year LPFS within treatment field was 68%. Local symptom improvement included palliation of bleeding (5/19 patients) and pain relief (6/19 patients). CONCLUSIONS: Electrochemotherapy may represent a new locoregional treatment for selected patients with superficial angiosarcomas. J. Surg. Oncol. 2016;114:246-253. © 2016 Wiley Periodicals, Inc.

Endoscopic Management of Recurrent Epistaxis Caused by Retiform Hemangioendothelioma in a Child: A Case Report.

Retiform hemangioendothelioma (RH) is a rare intermediate (locally aggressive) vascular tumor that mostly affects the dermis of the trunk and limbs, but has never been reported in the inferior turbinate. A 10-year-old Chinese boy presented with recurrent epistaxis in his left nasal cavity and anemia for more than 2 years. Radiographic and electronic video laryngoscopic images showed an expansile mass in the left inferior turbinate. Endoscopic surgery and electrocautery were performed to resect the tumor beyond the macroscopic border. Histopathologically, the tissues were infiltrated by hyperplastic blood vessels arranged in a retiform pattern, and endothelial cells proliferate significantly in some areas. Immunohistochemistry showed a positive result for CD31, CD34, Fli-1, and ERG. No epistaxis, tumor recurrence, or metastasis was found on reexamination over 18 months after surgery.

Multifocal lower limb hemangioendothelioma in a young female: a case report and review of the literature.

A 26-year-old female presented with pain and swelling of distal thigh and distal leg. She was diagnosed with multifocal epitheloid hemangioendothelioma (EHE) and was successfully treated with wide resection of femoral and tibial lesions followed by their reconstruction using vascularised fibular graft and local bone grafting. One year into follow-up, the patient remained asymptomatic with full Range Of Motion (ROM) and full weight bearing walking. This case illustrates a unique multifocal presentation of hemangioendothelioma and early surgical intervention leading to complete recovery, highlighting the importance of early diagnosis and intervention to help improve prognosis and quality of life of the patient.

Intravenous Lobular Capillary Hemangioma of the Neck: A Report of 2 Cases.

Background: Intravenous lobular capillary hemangioma (IVLCH) of the neck is a kind of rare benign tumor of vein. Purpose: In this paper, we report two female patients who were hospitalized because of neck masses. Results: The tumors in the neck veins of our patients were white oval masses with pedicle, clearly defined and of different sizes. Their immunohistochemical staining results showed CD31 (+), CD34 (+), SMA (+), ERG (+). The pathological diagnosis was intravenous lobular capillary hemangioma. Conclusions: Due to the location, morphology and immunohistochemistry, This lesion needs to be distinguished from other intravascular lesions such as thrombus, hemangiosarcoma and papillary endothelial hyperplasia.

Partially Dedifferentiated Primitive Malignant Melanoma with Pseudo-Angiomatous Features: A Case Report with Review of the Literature.

Malignant melanoma (MM) is traditionally known as the "great mime" of human pathology, as it is potentially capable of imitating the most disparate neoplasms. It is known that in addition to the more classic histotypes of MM, there are also rare forms, including angiomatoid MM. Similarly, it has been amply demonstrated in the literature that MM is capable of dedifferentiating, losing melanocytic lineage markers, constituting a diagnostic challenge for the pathologist. Although 5 cases of primary angiomatoid MM have been described in the literature, to the best of our knowledge, no cases of dedifferentiated melanoma with pseudo-angiomatoid aspects have ever been described. In this paper, we present a very rare case of partially dedifferentiated MM in which the most dedifferentiated component lost melanocytic lineage immunohistochemical markers and assumed a pseudo-angiomatous morphology. Given the rarity of the case, we carried out a literature review of similar cases described, trying to draw new future perspectives not only about this particular variant of MM but also about the widest field of dedifferentiation/undifferentiation of MM.

Propranolol for the treatment of infantile hemangiomas: a nine-year monocentric experience from a tertiary hospital.

BACKGROUND: Propranolol is currently considered the first-line therapy for problematic infantile hemangiomas (IH), the most common benign vascular neoplasm of infancy. OBJECTIVES: We present a retrospective observational study aimed at assessing the efficacy of propranolol in 44 IH patients. MATERIALS & METHODS: A nine-year retrospective review considering clinicodemographical and therapy-related variables was performed on medical records of infants treated for IH with oral propranolol. Each lesion was assessed through a numeric severity score based on size and colour both at baseline and after treatment conclusion (p <0.05 was considered statistically significant). RESULTS: Complete remission was achieved in 90.7% cases of IH with a general mean improvement in severity of 94.94%. No severe adverse effects were reported. Preterm patients showed a superior response compared to term infants, even though the difference was not significant (p=0.185). CONCLUSION: Propranolol showed high efficacy in terms of safety profile and cosmetic results. Prematurity and precocious therapy could be linked to a superior response.

Adult diffuse hepatic hemangiomatosis.

Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

An Uncommon Great Pretender in Oral Cavity Lesions: The Masson's Tumor.

Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign non-neoplastic vascular lesion. A typical presentation consists of a subcutaneous nodule that may simulate other clinical entities. Presentation in the oral cavity is uncommon. It is thought to develop as an abnormal proliferative reaction of endothelial cells in a process of impaired thrombogenesis. When endothelial proliferation occurs, a differential diagnosis with a soft tissue sarcoma, in particular an angiosarcoma, should be performed. We report a case of a 68-year-old female patient who presented with a lesion on the upper lip of 3 months' duration. Surgical resection revealed an IPEH. 1 year later, the patient showed a local recurrence requiring excision with clear margins. Pathological and immunohistochemical features can help us distinguish these lesions from those requiring more aggressive treatment. The gold standard is surgical resection with clear margins. Accurate preoperative diagnosis is essential to avoid overtreatment. Emphasis should be placed on clinical, radiological and histological studies.

Spindle cell haemangioma - Report of a rare entity.

Spindle cell haemangioma is a rare benign vascular neoplasm. Its occurrence in the oral cavity is uncommon and may be mistaken for other common oral lesions such as mucoceles or fibromas. Microscopically, it has features which are similar to those of other vascular neoplasms and may be a cause for potential mis-diagnosis. In this article, we report a case in the lower lip of a young male patient.