Reflectance confocal microscopy for Kaposi's sarcoma treatment monitoring after intralesional vincristine chemotherapy.

Kaposi's sarcoma (KS) is a multifocal systemic disease, originating from endothelial cells mainly affecting elderly men. Intralesional chemotherapy with vinblastine or vincristine is an effective and well-tolerated treatment in patients presenting single nodules on the skin. Despite reflectance confocal microscopy represents a useful diagnostic method for many dermatological diseases, to date, there are few data regarding the use of RCM in mucocutaneous KS. Objective of this study was to evaluate the use of RCM for therapeutic follow-up in KS patients treated with intralesional vincristine. An observational retrospective study involving patients with a histological diagnosis of classic KS was conducted. All patients were treated with intralesional vincristine; reflectance confocal microscopy images were taken for each patient at baseline (T0) and 1 month after vincristine injection. Four male patients with a median age of 76.8 years were included in the study and four nodules (one for each patient) were evaluated with RCM examination before and after vincristine injections. At 1 month from intralesional vincristine treatment, therapeutic response was confirmed at RCM examination; a reduction of inflammatory cell at the stratum spinosum level in all evaluated lesions was observed; at papillary dermis levels, black luminal structures were decreased in diameter and superficial linear canalicular structures were not represented. Aggregates of inflammatory cells and of hemosiderin deposition, at the dermal level, were reduced in number. Reflectance confocal microscopy showed to be a promising method to evaluate vincristine therapeutic response in patients with KS; further studies evaluating RCM use in KS patients in order to monitor treatment efficacy are still required.

Management of Refractory Mandibular Kaposiform Hemangioendothelioma with Sirolimus: A Case Report and Review of the Literature.

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of intermediate malignancy that generally occurs in infancy and early childhood. Typically, the lesion arises from superficial or deep soft tissues of the extremities, trunk and retroperitoneum. The paucity of reported cases of head and neck KHEs is evidence of the rarity of the disease in this region. We report on the presentation and treatment of KHE in an 11-month-old boy who presented with a mandibular lesion. We include a brief discussion about the differential diagnosis of KHE. Management involved preoperative interventional radiology, surgical excision and chemotherapeutic treatment with Sirolimus. The lesion resolved without evidence of relapse 12 months later.

A Review of Viral-Related Malignancies and the Associated Imaging Findings.

OBJECTIVE. Imaging plays an important role in the diagnosis and staging of malignancies. Many common lymphoproliferative and other solid tumor malignancies can be viral-related. CONCLUSION. This review discusses the imaging findings that can be associated with common viral-induced malignancies. Knowledge of these imaging presentations can help narrow the differential diagnosis to reach a specific diagnosis through a precise workup and proper management.

Ultrasonographic intraoperative monitoring and follow-up of Kaposi's sarcoma nodules under treatment with intralesional vincristine.

BACKGROUND: Intralesional vincristine is an effective treatment for Kaposi's sarcoma (KS) nodules on the skin, but there is little evidence of its action through imaging techniques. Ultrasonography can be an adjunctive tool in the diagnosis and management of KS skin lesions, but data in the literature are few. MATERIALS AND METHODS: Five patients with classic KS nodules were treated with intralesional vincristine. Ultrasonographic and color Doppler assessment were performed during vincristine injection and monitoring was repeated 1 and 3 months after the procedure. Partial response was defined as a reduction of more than 50% lesion volume and reduction of the vascular signal; complete response as a resolution of lesion associated with the absence of vascular signal. RESULTS: Six KS nodules were included in the study. On ultrasonography examination, KS nodules appeared as oval or round, hypoechoic, homogeneous structures, with intralesional vascularization, more prominent in the deepest pole of the nodule. At month 1, 4 nodules achieved a complete response, while two nodules showed a partial response and were retreated with intralesional vincristine. At month 3, all lesions achieved a complete response. CONCLUSION: Ultrasonography may be a valuable tool in assessing clinical response to intralesional vincristine therapy of cutaneous KS nodules.

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

CASE REPORT: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child's condition has markedly improved. DISCUSSION AND CONCLUSIONS: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child's pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Kaposiform Hemangioendothelioma Presenting as Hydrops Fetalis.

We describe the case of a 33-week preterm infant who developed nonimmune hydrops fetalis secondary to a kaposiform hemangioendothelioma (KHE). The tumor was successfully treated with vincristine, prednisone, ticlopidine, and aspirin. KHE can be an unusual cause of hydrops fetalis; in such cases, diagnosis can be challenging since generalized edema can obscure KHE.

Imaging features of kaposiform lymphangiomatosis.

BACKGROUND: Kaposiform lymphangiomatosis is a rare, aggressive lymphatic disorder. The imaging and presenting features of kaposiform lymphangiomatosis can overlap with those of central conducting lymphatic anomaly and generalized lymphatic anomaly. OBJECTIVE: To analyze the imaging findings of kaposiform lymphangiomatosis disorder and highlight features most suggestive of this diagnosis. MATERIALS AND METHODS: We retrospectively identified and characterized 20 children and young adults with histopathological diagnosis of kaposiform lymphangiomatosis and radiologic imaging referred to the vascular anomalies center between 1995 and 2015. RESULTS: The median age at onset was 6.5 years (range 3 months to 27 years). The most common presenting features were respiratory compromise (dyspnea, cough, chest pain; 55.5%), swelling/mass (25%), bleeding (15%) and fracture (5%). The thoracic cavity was involved in all patients; all patients had mediastinal involvement followed by lung parenchymal disease (90%) and pleural (85%) and pericardial (50%) effusions. The most common extra-thoracic sites of disease were the retroperitoneum (80%), bone (60%), abdominal viscera (55%) and muscles (45%). There was characteristic enhancing and infiltrative soft-tissue thickening in the mediastinum and retroperitoneum extending along the lymphatic distribution. CONCLUSION: Kaposiform lymphangiomatosis has overlapping imaging features with central conducting lymphatic anomaly and generalized lymphatic anomaly. Presence of mediastinal or retroperitoneal enhancing and infiltrative soft-tissue disease along the lymphatic distribution, hemorrhagic effusions and moderate thrombocytopenia (50-100,000/µl) should favor diagnosis of kaposiform lymphangiomatosis.

18F-fluorodeoxyglucose Positron Emission Tomography in Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease: Correlation With Activity, Severity, Inflammatory and Virologic Parameters.

BACKGROUND: Kaposi sarcoma herpesvirus (KSHV)-associated multicentric Castleman disease (MCD) is a lymphoproliferative inflammatory disorder commonly associated with human immunodeficiency virus (HIV). Its presentation may be difficult to distinguish from HIV and its complications, including lymphoma. Novel imaging strategies could address these problems. METHODS: We prospectively characterized (18)F-fluorodeoxyglucose positron emission tomography (PET) findings in 27 patients with KSHV-MCD. Patients were imaged with disease activity and at remission with scans evaluated blind to clinical status. Symptoms, C-reactive protein level, and HIV and KSHV loads were assessed in relation to imaging findings. RESULTS: KSHV-MCD activity was associated with hypermetabolic symmetric lymphadenopathy (median maximal standardized uptake value [SUVmax], 6.0; range, 2.0-8.0) and splenomegaly (3.4; 1.2-11.0), with increased metabolism also noted in the marrow (2.1; range, 1.0-3.5) and salivary glands (3.0; range, 2.0-6.0). The (18)F-fluorodeoxyglucose PET abnormalities improved at remission, with significant SUVmax decreases in the lymph nodes (P = .004), spleen (P = .008), marrow (P = .004), and salivary glands (P = .004). Nodal SUVmax correlated with symptom severity (P = .005), C-reactive protein level (R = 0.62; P = .004), and KSHV load (R = 0.54; P = .02) but not HIV load (P = .52). CONCLUSIONS: KSHV-MCD activity is associated with (18)F-FDG PET abnormalities of the lymph nodes, spleen, marrow, and salivary glands. These findings have clinical implications for the diagnosis and monitoring of KSHV-MCD and shed light on its pathobiologic mechanism.

Skin Ultrasound in Kaposi Sarcoma. / Ecografía cutánea en el sarcoma de Kaposi.

The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

Fine-needle aspiration cytology of disseminated Kaposi sarcoma of the bone in an AIDS patient.

BACKGROUND: Kaposi sarcoma (KS) is a vascular neoplasm associated with human herpesvirus 8 (HHV-8). Skin and mucous membranes are the most common sites, but other organs may be involved. Skeletal KS is rare and occurs either by direct spread of mucocutaneous lesions or through dissemination. Patients present with bone pain and lytic lesions for which they may undergo fine-needle aspiration (FNA). While there are about 70 published case reports of skeletal KS, there is limited literature specifically describing its cytomorphology. Our literature search yielded only a single prior reported case of FNA biopsy of skeletal KS in a Nigerian AIDS patient. CASE: We present a case of disseminated KS of the axial skeleton in a 45-year-old African-American man with AIDS which was diagnosed on FNA cytologic examination. The patient presented with multiple lytic lesions in the axial skeleton. The aspirate, core-needle biopsy and touch imprint cytology of a bone lesion demonstrated clusters of spindle and epithelioid cells in radial and streaming arrangement with indistinct intercytoplasmic borders, elongated nuclei, fine chromatin and inconspicuous nucleoli. Immunohistochemical studies revealed positivity for HHV-8 and vascular markers. The cytomorphologic and ancillary features of the case are presented and discussed.

Computed tomography virtual bronchoscopy: normal variants, pitfalls, and spectrum of common and rare pathology.

A broad spectrum of pathologies that involve the laryngotracheobronchial airway and imaging plays a crucial role in evaluating these abnormalities. Computed tomography with virtual bronchoscopy has been found to be very helpful in defining the location, extent, and nature of these lesions, and is increasingly being used even in patients with contraindications for fiberoptic bronchoscopy and laryngoscopy. Ionizing radiation, associated with virtual bronchoscopy, can be minimized by using low-dose multidetector computed tomography and hybrid iterative reconstruction techniques. Furthermore, retrospectively generated virtual bronchoscopy from a routinely acquired computed tomography data set eliminates additional cost and radiation. In the future, virtual bronchoscopy assisted with advanced navigational techniques will broaden the diagnostic and therapeutic landscape. This article presents the characteristic features of common and rare laryngotracheobronchial pathologies seen with virtual bronchoscopy.