BACKGROUND: Retroperitoneal infantile hemangioma (RIH), a type of primary retroperitoneal tumors, are exceptionally rare in clinical practice. Infantile hemangiomas typically manifest on the skin\'s surface. RIHs are exceptionally rare and typically small. In adults, these tumors often manifest without specific clinical symptoms or detectable signs for a definitive diagnosis. This case report details a patient diagnosed with RIH. We recommend complete excision of the tumor after a comprehensive evaluation, followed by postoperative pathology, to achieve a conclusive diagnosis. We believe that managing critical retroperitoneal structures and vessels intraoperatively presents a significant challenge for all procedures involving primary retroperitoneal tumors. A 47-year-old male was diagnosed with gallstones and underwent surgery 3 months ago at other institution for unexplained nausea and vomiting. Follow-up imaging 2 months after surgery revealed a retroperitoneal mass below the left renal pole. Upon presentation to our hospital, the patient continued to experience intermittent nausea and vomiting, with no other significant symptoms or signs. Considering the patient\'s 8-year history of hypertension, a paraganglioma was initially suspected. We performed the laparoscopic mass resection after a detailed assessment. However, postoperative pathology revealed it a capillary hemangioma (old term)/infantile hemangioma.
CONCLUSIONS: RIHs are exceedingly rare benign tumor. The possibility of malignancy should be ruled out, and surgical resection is recommended following a thorough evaluation, with the diagnosis confirmed through pathological examination.

BACKGROUND: Papillary intralymphatic angioendothelioma (PILA) is an exceptionally rare metastasizing soft tissue tumor. It tends to arise in the subcutaneous tissues of distal extremities in children. Only four intraosseous PILA cases have been reported until now in English language literature.
METHODS: We present a case of PILA arising in the distal femoral epiphysis of a 50-year-old female patient. It started as a relentless pain in her left knee. A plain radiography revealed a radiolucent area in the left internal femoral condyle. Computerized tomography revealed a 1-cm lytic lesion with a sclerotic rim. Magnetic resonance images showed a significant bone marrow edema signal focused on a 1-cm subchondral lesion suggestive of an intraarticular osteoid osteoma. Histologically, the tumor contained vascular channels covered by a single endothelial layer with intraluminal papillary endothelial structures lined with hobnail cells. Immunohistochemically, the cells were positive for ERG, CD31, and D2-40. The tumor underwent cryoablation and 6 months later, after local recurrence or tumor persistence, a wide tumor resection was referred. After 7 years of follow-up, the patient displayed neither local recurrence nor distant metastases.
CONCLUSIONS: Primary intraosseous PILAs are exceedingly rare tumors that should be considered in the differential diagnosis of vascular bone tumors.

Hemangiomas are benign vascular tumors and are classified into cavernous, capillary, and mixed, with the head and neck area as the most common site. Hemangiomas are common in pediatrics and rare in adults. Diagnosing cavernous hemangioma is challenging and requires a complete history, proper physical examination, and several radiological modalities to improve diagnostic accuracy because it is uncommon in adults. Herein, we present a case of a 66-year-old female Saudi patient with cavernous hemangioma from the diagnosis until the surgical treatment. No previous studies are reported in Saudi Arabia and this is a rare presentation of cavernous hemangioma at this age. Cavernous hemangioma in the parotid gland in adults is uncommon and is difficult to diagnose. Therefore, a thorough physical examination and several radiological modalities are required to improve diagnostic accuracy. The most effective treatment of cavernous hemangioma in adults is surgical resection.

UNASSIGNED: Anastomotic hemangioma is a rare subtype of capillary hemangioma primarily found in the genitourinary tract. We present a case of a patient with an anastomotic hemangioma located in the retroperitoneal space; then, we explore and summarize the imaging features from previously reported cases for accurate diagnosis.
UNASSIGNED: A 57-year-old woman complained of left lower back pain. Contrast-enhanced ultrasound revealed a hypoechoic mass with \"slow-in and slow-out\" enhancement. Abdominal CT scan displayed a well-defined, round soft tissue mass in the right retroperitoneal region with obvious enhancement. MRI indicated low signal on T1-weighted imaging, high signal on T2-weighted imaging and diffusion-weighted imaging, and progressive enhancement after enhancement. Surgical removal of the tumor was performed. Histopathological examination exhibited a distinct tumor border with interconnected blood vessels and a cavity lined by a single layer of cubic endothelial cells. Immunohistochemistry confirmed the presence of CD31[+] and CD34[+]. The final pathological diagnosis was anastomotic hemangioma. No recurrence was observed during a 40-month follow-up.
UNASSIGNED: Retroperitoneal anastomotic hemangioma is a rare and benign neoplasm that may be misdiagnosed as ectopic pheochromocytoma or angiosarcoma. This case report presents and analyzes the imaging characteristics of a series of retroperitoneal anastomotic hemangiomas, which can be valuable for future diagnoses and help prevent unnecessary surgeries.

UNASSIGNED: The classification of vascular anomalies includes terms of nomenclature that are not based on histogenesis resulting in confusion among health professionals of different specialties. Ongoing efforts to classify them properly have taken place. This literature review aimed to identify erroneous nomenclature of vascular anomalies and to investigate their continued use over the past four years after the last International Society for the Study of Vascular Anomalies (ISSVA) update.
UNASSIGNED: Literature research was based on pertinent classifications (ISSVA, WHO) and books related to vascular anomalies and soft tissue pathology. After identifying twelve entities with confusing terminology, new research in the Pubmed database was conducted to verify their continued use in the last four years.
UNASSIGNED: The literature review highlighted terms referring to vascular malformations as neoplasms. In addition, terms used as equivalents represent entirely different entities. On the other hand, different terms to characterize the same entity were also recorded. Furthermore, regardless of the last ISSVA update in 2018, terms that are only descriptive or do not correspond to vascular anomaly histogenesis are consistently used.
UNASSIGNED: Despite intensive efforts in the last decades for correct terminology and classification of vascular anomalies, modifications are still required. A common and broadly accepted scientific terminology should be applied, accurately representing histogenesis or pathogenesis, to obtain a common language among medical specialists, given that a multidisciplinary approach is crucial for managing vascular anomalies. HIPPOKRATIA 2022, 26 (4):126-130.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors.
METHODS: We conducted a literature search from 1 January 2000, to 30 April 2022. Articles reporting outcomes of patients treated with mTOR inhibitors for KHE were included. Descriptive statistics were used to describe and summarize the outcomes of the treatment.
RESULTS: We included 327 patients with a mean age at diagnosis of 9.1 months (SD ± 9). Patients were treated with an mTOR inhibitor for a mean of 15.2 months (SD ± 4.1). A total of 315 (96.3%) patients had positive outcomes including improvement of the tumor size, symptoms and/or laboratory parameters in 227 (85%) and complete remission in 38 (12%). Seven (2%) patients did not respond to treatment and seven (2%) died of sepsis (4), Kasabach-Merritt phenomenon complications (1), cardiac and liver failure due to ductus arteriosus (1), or metastatic disease (1).
CONCLUSIONS: This systematic review supports the efficacy and safety of mTOR inhibitors for KHE. Their use resulted in positive outcomes in terms of decreased symptoms, reduction in tumor size and improvement in biochemical parameters with a mortality rate of 2%. According to these findings, we suggest revised consensus treatment guidelines for KHE with mTOR inhibitors potentially considered first-line therapy.

Although hemangiomas are common in the head and neck area, they are uncommon in the temporal bone. External auditory canal (EAC) hemangiomas are a relatively uncommon otologic condition. In the English literature, only 29 cases of temporal bone hemangioma have been reported. We also present the case of a 32-year-old male patient who presented with an 8-month history of decreased hearing, tinnitus, and aural fullness on the right side. Otoscopic examination of the right ear revealed a smooth, oval, well-circumscribed, reddish, pulsatile, nontender mass, which was about 1 cm in diameter and occupied two-thirds of the EAC. It was soft in consistency, compressible, and appeared to be arising from the right posterior-superior portion of the bony canal wall; the tympanic membrane seemed to be uninvolved. The patient had mild conductive hearing loss of the right ear with an average air-bone gap of 20 dB; the left side was normal. High-resolution contrast-enhanced computed tomographic scanning of the temporal bone showed well-defined, rounded, homogenously enhancing lesions at the posterior-superior aspect of right EAC measuring 0.7 × 0.8 cm. Angiography was performed and there was evidence of vascular blush in the region of the right EAC. Three blood vessels were identified and embolized. The mass was completely removed by the endaural approach, and a retroauricular skin graft to restore cutaneous integrity was not required. Histopathology indicated a capillary hemangioma without cytological atypia or mitotic activity. There was no recurrence 1 year after the surgery.

In addition to the evaluation of melanocytic growths (Part I), dermoscopy is helpful in the identification and management of vascular tumors, skin infections, and inflammatory conditions. In this practical review, we present the classic dermoscopic findings of the following: vascular tumors, infectious conditions (molluscum contagiosum, scabies, verruca vulgaris), inflammatory conditions (psoriasis, atopic dermatitis), juvenile xanthogranuloma, and nevus sebaceus.

Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management.

The management of vascular anomalies is complex and requires a multidisciplinary team with a combination of medical, surgical, and intervention treatments. Medical treatment is limited and has conflicting results. Off-label use of mammalian target of rapamycin inhibitors shows promising results. The objective of this study was to systematically evaluate the literature published about the efficacy and safety of sirolimus in the treatment of vascular anomalies.
A systematic review of the published literature was conducted using the PubMed database and Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
There were 73 articles included: 2 randomized controlled studies, 2 nonrandomized prospective studies, and 69 retrospective case reports and case series. In total, 373 patients were included. Sirolimus was administered topically to 56 patients and orally to 317 patients. Sirolimus was highly effective in the treatment of vascular tumors associated with Kasabach-Merritt phenomenon (95.5% of the patients clinically improved and 93% had normalization of coagulopathy), venous malformations (size reduction was observed in 88.9% of patients), and lymphatic malformations (clinical improvement in 94.9% of patients). Topical sirolimus results were conflicting. Arteriovenous malformations were not improved by sirolimus.
Low-level evidence suggests that sirolimus can improve the prognosis of vascular anomalies, most notably vascular tumors associated with life-threatening coagulopathy and venous and lymphatic malformations. Further research is needed to establish the benefits of sirolimus in the management of vascular anomalies.