Atypical Vascular Proliferation Research Articles

Novel Manifestation of Retinal Hemangioblastomas Detected by OCT Angiography in von Hippel-Lindau Disease

To elucidate the clinical characteristics of atypical retinal vascular proliferation in patients with von Hippel-Lindau (VHL) disease using OCT angiography (OCTA). Prospective, observational study. Fifty-seven consecutive patients with a diagnosis of VHL disease who visited Kyoto University Hospital between January 2019 and March2022. Retinal hemangioblastomas (RHs) were assessed using multimodal imaging including OCTA. Retinal hemangioblastomas were classified into 2 phenotypes: nodular and flat. Nodular RHs were defined as typical RHs that were globular, well-circumscribed tumors, often accompanied with dilated feeder arterioles and draining venules. Flat RHs lacked a protruded red or colored mass, had variable and indistinct borders, and were not accompanied with feeder and draining vessels. The prevalence, distribution, and description of atypical flat RHs. Among 57 consecutive patients with VHL disease, 37 patients (64.9%) showed RHs in at least 1 eye. Bilateral RHs were seen in 23 patients (62.2%). Among 58 eyes of 37 patients with RHs, typical nodular RHs were detected in 54 eyes. Nodular RHs were seen mainly in the peripheral retina and occasionally in the peripapillary region, and they showed exudative changes in some cases. Flat RHs were detected in 7 eyes (12.1%). Four eyes showed only flat RHs, and 3 eyes showed both types in the same eye. Most flat RHs appeared as retinal hemorrhages or faint flat abnormal retinal vessels in the inner retina on the fundus examination, often within the macula area or peripapillary. In all eyes with flat RHs, OCTA showed abundant blood flow in the lesions. OCT revealed that flat RHs were seen mainly between the retinal nerve fiber layer and the ganglion cell layer, and occasionally within the inner nuclear layer. During a mean follow-up period of 20.4 ± 15.0 months, no flat RHs accompanied exudative change, tractional retinal detachment, or progression in size. Patients with VHL disease can demonstrate 2 distinct types of RHs: the classic nodular type and an atypical flat type. OCT angiography can be useful in improving the detection of atypical flat RHs, which can be difficult to detect clinically. Proprietary or commercial disclosure may be found after the references.

Kaposi sarcoma

A 47-year-old heterosexual man with history of intravenous (IV) methamphetamine use presented to the Emergency Department with dyspnea and raised lesions throughout his arms, legs, and chest as depicted in Figure 1. Biopsy of a right leg lesion demonstrated atypical vascular proliferation within the dermis producing slit-like/irregular architecture consistent with Kaposi sarcoma. Human herpesvirus 8 (HHV-8) staining was consistent with Kaposi sarcoma. HIV-1 antibody testing was positive and the patient had an absolute CD4 count of 19. Kaposi sarcoma is relatively rare in the United States as antiretroviral therapy has become more accessible. The appearance of a vascular tumor with inflammation and mucosal involvement should raise suspicion for HHV-8.

Clinicopathologic and immunohistochemical study of breast angiosarcoma

Breast angiosarcoma (AS) is a rare malignancy which can be classified into primary or secondary as a result of breast cancer therapy. On histology, breast AS has a wide spectrum of morphologic presentations, and its diagnosis can be challenging based on morphologic evaluation alone. Here, we studied 10 cases of breast AS diagnosed at our institution during a 20-year period, in which 7 cases were radiation-associated AS (RA-AS) and 3 cases were primary AS (P-AS). The average latency between radiotherapy and RA-AS was 8.1 years. RA-AS mostly occurred in breast skin, while all P-AS involved breast parenchyma. All 10 AS cases were high grade, including 4 RA-AS cases demonstrating epithelioid morphology. Histologic morphologies of AS varied from confluent growth of atypical spindle or epithelioid cells to scattered marked pleomorphic cells. Some cases appeared deceptively bland or low grade, but the presence of areas of haemorrhage (‘blood lake’) or necrosis upgraded them to high grade lesions. Additionally, some epithelioid RA-AS cases with lymphatic differentiation (D2–40 positive) showed pseudopapillary morphology characterized by discohesive cells sloughing off at periphery of vascular cores, resembling papillary breast carcinoma. P-AS did not show prominent vesicular nuclei and/or conspicuous nucleoli, which were features observed in RA-AS. C-MYC immunostain results showed P-AS was completely negative or focal weakly positive in hypercellular areas. In comparison, RA-AS were consistently positive for c-MYC. Epithelioid RA-AS with lymphatic differentiation tended to show stronger and/or more diffuse c-MYC positivity than other AS cases. CD31 and ERG immunostains showed positivity in all cases, while CD34 were negative in some cases with lymphatic differentiation. This study offers a detailed morphologic and immunohistochemical assessment of a rare tumor of the breast that is important to recognize. Common differential diagnosis for breast AS, including post-radiation atypical vascular proliferation (AVP), are also reviewed and discussed.

GORHAM-STOUT DISEASE IN THE MANDIBLE: A CASE REPORT

<h3>Introduction</h3> Gorham-Stout disease is a rare disease that was first described by Jackson in 1838. The first case in jaws was reported in 1924. There is no clear consensus on the etiopathogenesis of the disease. A few proposed theories include bone destruction secondary to hyperemia from hemangiomatosis, increased osteoclastic activity mediated by IL-6, and lymphangiogenesis mediated by vascular endothelial growth factor, platelet-derived growth factor, and IL-6. The disease presents as an osteolytic lesion without cortical expansion. Here, we present a case of Gorham-Stout disease exhibiting hemorrhagic gingival overgrowth in the mandible. <h3>Case Report</h3> A 46-year-old female patient presented with a chief complaint of blisters and bubbles in her lower gums. Clinical examination revealed multiple loose teeth in the anterior and left mandible with hemorrhagic and hyperplastic gingival tissue. Upon radiographic evaluation, significant bone loss was recognized in the area, giving the appearance of "teeth floating in air." The differential diagnosis included Gorham-Stout disease, osteomyelitis, Langerhans cell histiocytosis, and other conditions with osteolytic changes. Histologic examination of the biopsy specimen revealed atypical vascular proliferation with mixed acute and chronic inflammation favoring exuberant granulation tissue. Her lab workup confirmed elevated levels of serum IL-6 and vascular endothelial growth factor. A diagnosis of Gorham-Stout disease was made based on these findings. The patient presented with a pathologic fracture of her left mandible 3 months after her initial consultation. She was referred to rheumatology and treatment with bisphosphonates was initiated. However, the patient failed to return for further treatment with the physician and was lost to follow-up.

687. Iatrogenic Kaposi’s Sarcoma Following Treatment with Vedolizumab

BackgroundKaposi’s sarcoma (KS) is a vascular tumor caused by human herpes virus-8 infection (HHV-8) commonly involving the skin. We report a case of a patient with controlled HIV who developed Kaposi’s sarcoma on vedolizumab.Darkened hyperkeratotic plaque on his left medial foot MethodsA 39-year-old homosexual male with a history of Ulcerative Colitis (UC), Hodgkin’s lymphoma in remission and HIV presented with complaints of abdominal pain and bloody diarrhea. He had new tender lesions on his left foot which was absent at his previous admission a month ago. The lesions started as macules and later progressed to tender lesions. Mesalamine and oral corticosteroids were previously prescribed without symptomatic relief and he was started on vedolizumab 2 months ago. On physical exam he had abdominal tenderness, tender cervical and inguinal lymph nodes, and dark macules on his feet. Digital rectal exam revealed bloody mucoid stool. Laboratory showed white blood cell count of 12,600/mm3, ESR of 132 mm/hr and CRP of 4.6 mg/dL. His CD4 T-cell count was 873 cells/mm3 and viral load was 50 copies per milliliter. Cervical lymph node biopsy showed polymorphous population of lymphocytes but was negative for malignant cells. Biopsy of the foot plaques showed atypical intradermal vascular and spindle cell proliferation positive for HHV-8 and for vascular marker CD34. The results were consistent with the diagnosis of KS. Serology was also positive for HHV-8 with high viral titers of 74 copies/mL. Colonoscopy showed severe proctitis with deep ulcerations in a continuous pattern in the rectum with a normal sigmoid colon. Follow up colonoscopy showed improved proctitis and he was started on doxorubicin to treat KS with improvement of the foot lesion a month later following treatment.Dark macules on the sole of both feet Figure 3a: Spindle cells with irregular small vessel proliferation and red blood cell extravasation between tumor cells Figure 3b: Immunohistochemical stain showing HHV-8 expression of spindle cells Improvement of the foot lesion ResultsVedolizumab is a monoclonal antibody that prevents the recruitment of lymphocytes to the inflamed tissue. It is approved for the treatment of IBD and has shown efficacy and safety. The iatrogenic form of Kaposi’s sarcoma occurs in patients on immunosuppressive therapy, as this patient.ConclusionPatients with IBD on immunosuppressive drugs should be followed up closely and screened for latent viral infections prior to initiating therapy. As in the patient, HHV-8 should be recognized as a likely underlying opportunistic infection in immunocompromised patients with IBD.Disclosures All Authors: No reported disclosures

Pulmonary Epithelioid Haemangioendothelioma Involving Pleura: A Case Report

Abstract Introduction/Objective Pulmonary epithelioid haemangioendothelioma is a rare, low to intermediate grade tumor of endothelial origin. It is more common in middle aged women. In lung, more than 60 % of the cases present as bilateral multiple perivascular nodules on imaging raising the differential of metastasis or inflammatory cause. WWTR1- CAMTA1 fusion gene is characteristic for this entity. Evidence of pleural involvement carries a poor prognosis. Methods Here, we present a case of epithelioid haemangioendothelioma involving pleura in a 52-year-old woman with a long standing history of rheumatoid arthritis. Patient presented with recurrent pleural effusions and shortness of breath. Results A diagnosis of epithelioid haemangioendothelioma was made on her pleural decortication specimen that showed atypical vascular proliferation and confirmed by immunohistochemical staining. Subsequent right lung wedge resection performed later confirmed the presence of epithelioid haemangioendothelioma in lung parenchyma. Conclusion Pathologists should be aware of this rare condition that can clinically present in a very deceptive manner of recurrent effusion.

The Vascular Convolutions-Papillary Endothelial Hyperplasia

Intravascular papillary endothelial hyperplasia is an exceptional, benign, inflammatory, vascular neoplasm delineating papillary configuration engendered from reactive proliferation of damaged endothelial cells, while being confined to a thrombus. Initially scripted by Pierre Mason in 1923, the tumefaction was denominated as an intra-luminal lesion within an ulcerated, haemorrhoidal vein and designated as “hemangio-endotheliome’ vegetant’ intravasculaire”(1). The neoplasm is additionally nomenclated as Masson’s tumour, Masson’s pseudo-angiosarcoma, endovascularite proliferante thrombopoietique, intravenous atypical vascular proliferation, intravascular angiomatosis, vascular angiomatosis, intravascular endothelial proliferation, reactive papillary endothelial hyperplasia or intravascular papillary endothelial hyperplasia. The papillary neoplasm is associated with deposition of fibrin and thrombotic substances within a painful, ulcerated.

Atypical Vascular Proliferation Secondary to Radiotherapy in a Patient With a History of Synovial Sarcoma

We present the case of a 21-year-old male patient with a history of monophasic synovial sarcoma in his left thigh, which was treated with surgical resection, radiotherapy, and chemotherapy with mesna, doxorubicin, and Ifosfamide (MAI protocol). Approximately six years after the end of the oncological treatment, he presented a nodular, polypoid lesion in the left popliteal region, which was painless and fast growing. Ultimately, the biopsy was consistent with atypical vascular proliferation (AVP). Vascular lesions after radiotherapy include a wide spectrum of pathologies that range from benign lesions such as AVP to malignant ones with very poor prognosis such as angiosarcoma, the distinction between one and the other can be difficult, being the determination rearrangement or amplification of gene c-myc, a key to make an accurate diagnosis in case of doubt.

Atypical vascular proliferations of the neck.

Atypical vascular proliferations (AVP) are alate complication after radiotherapy. Most cases have been reported in female breast cancer patients on the chest wall. These lesions are mostly of the lymphatic type. Herein, we report ablood vascular-type AVP in amale on the neck 60years after radiotherapy for abenign hemangioma, which makes this case exceptional. We removed the whole chronic radiodermatitis surgically. Histopathology excluded vascular malignancies but confirmed AVP. We discuss the differential diagnoses and treatment.

Characteristics of atypical postradiation vascular proliferation: A retrospective review of 193 patients

Characteristics of atypical postradiation vascular proliferation: A retrospective review of 193 patients

240 Characteristics of atypical post-radiation vascular proliferation: a multicenter retrospective review

240 Characteristics of atypical post-radiation vascular proliferation: a multicenter retrospective review

Atypical vascular proliferation after radiation for breast carcinoma atypical vascular proliferation

We present the case of a year old Caucasian woman with a history of stage II invasive ductal carcinoma of the left breast that presented atypical erythematous lesions of three months history after radiation two years before for a breast carcinoma Biopsy was done to distinguish a benign lesion from the main differential diagnosis which is angiosarcoma Distinguishing post radiation benign from malignant vascular lesions can be challenging because they share overlapping clinical and histopathologic features Thus any vascular lesion that occurs in a previously irradiated skin should be excised completely with tumor free margins and examined histologically Prolonged follow ups after radiation should be carried on in order to be aware that a lesion in the beginning which was benign could be transformed into post radiation angiosarcoma further on in time There is some evidence that they represent a precursor to radiation induced angiosarcoma

Masson's Tumor Presenting as an Asymptomatic Nodule on Lower Lip in a 30-Year-Old Male.

Sir, Masson's tumor, also known as intravascular papillary endothelial hyperplasia (IPEH), is a benign tumor of the skin comprising reactive hyperplasia of intravascular endothelial cells in the vicinity of an organized thrombus. This tumor was first described by Masson in 1923 and was named as hemangioendotheliome vegetant intravasculaire. Since then, it has been variously named as endovascularite proliferante thrombopoietique, intravenous atypical vascular proliferation, intravascular angiomatosis, etc.[1] Our case is of a 30-year-old male who presented with an asymptomatic erythematous papule on the lower border of the lower lip since 13 years. He did not complain of any discomfort due to the swelling, however, on pricking the lesion with a common pin there was oozing of blood, which he did purposefully on one or two occasions in an attempt to get rid of it. On examination, the lesion was soft in consistency and nontender. The surrounding area was normal. Submental and submandibular lymph nodes were not enlarged. Differential diagnoses of mucous cyst, vascular polyp, and venous lake were kept [Figure 1].Figure 1: A papule visible at the lower border of lower lipThe lesion was surgically excised and sent for histopathological examination, which revealed a dilated vascular space in the submucosa with an organized thrombus and papillary hyperplasia of endothelial cells on one side. A final diagnosis of IPEH or Masson's tumor in a venous lake of lip was made [Figures 2 and 3].Figure 2: An organized thrombus within a dilated vascular space [Hematoxylin and eosin, ×100]Figure 3: At one side of the thrombus there is a proliferation of endothelial cells within the dilated vascular space [Hematoxylin and eosin, ×400]IPEH is a reactive phenomenon occurring usually in association with a venous dilatation or any other vascular channel.[1] Hashimoto[2] classified this entity into three types as “pure,” “mixed,” and “extravascular.” Pure type (55.8%) arises de novo in a dilated vascular space with no causative comorbidity. Mixed (39.9%) type occurs superimposed over a pre-existing vascular anomaly such as arteriovenous malformations and hemangiomas associated with venous thrombosis. Extravascular type (4.3%) is primarily associated with trauma induced hematoma formation. Our case falls in the category of mixed type of IPEH as it was in the vicinity of an organized thrombus. Histopathology of this lesion is classical with masses of papillary structures associated with a thrombus within the lumen of a vessel. Each papillary frond has fibrin or collagenous connective tissue as its core and is lined by plump endothelial cells. Mitotic figures may be present. Atypia and necrosis may or may not be present.[2] The pathogenesis of this tumor is debatable. Most favoured considerations include benign neoplastic process, with endothelial cell proliferation and papillary formation in the vascular lumen undergoing degeneration and necrosis in the manner of a red infarct, a reactive process of endothelial cells induced by blood stasis and perivascular inflammation, a benign endothelial proliferation arising from a thrombus as a variant of angiolymphoid hyperplasia with eosinophilia, and a pseudotumoral lesion caused by endothelial proliferation with papillary formation preceeded by a thrombotic material, which serves as a developmental material.[3] Closest histopathological differential of IPEH is angiosarcoma.[4] The characteristics of IPEH which differentiates it from angiosarcoma include circumscribed lesion, intraluminal location, papillary formation related to a thrombus, fibrohyalinized core of papillae, one or two layers of endothelial cells, hyperchromatic endothelial cells may be present, not true endothelial fronds, uncommon piling up of endothelium, obscure cellular pleomorphism, rare mitotic activity, rare necrosis, along with absence of irregular capillary vessels.[5] Masson's tumor is more frequent in females, and very few cases have been reported in and around the oral cavity, with the most common location being the lower lip and the least being the angle of the mouth.[4] Treatment is surgical excision with healthy margins. We report this case since Masson's tumor in venous lake of lip is not a very common entity. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

A Case of Patch Stage of Kaposi's Sarcoma and Discussion of the Differential Diagnosis

A 55 year old HIV positive male had a skin lesion biopsy which showed atypical vascular proliferation within the superficial and deep dermis with mild atypia of lining endothelial cells. A sparse lymphoplasmacytic infiltrate surrounding the irregular vascular channels was noted. Immunohistochemistry highlighted the atypical blood vessels with the vascular markers CD31, CD34 and Factor VIII. The differential diagnosis included unusual vascular or lymphatic proliferations, stasis dermatitis, kaposiform hemangioendothelioma, progressive lymphangioma and angiosarcoma with focal Kaposi’s sarcoma features. Characteristic human herpes virus-8 positive staining helped support the diagnosis of patch stage of Kaposi’s sarcoma. Herein, we discuss the case findings, differential diagnosis and characteristic histological findings associated with the patch stage of Kaposi’s sarcoma which can be an elusive diagnosis.

Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases.

Primary angiosarcoma of the bladder is very rare, with approximately 30 cases reported in the literature. Those with epithelioid morphology are even rarer, with only single-case reports published. We describe the histopathologic features and clinical follow-up of 9 patients with epithelioid angiosarcoma (EA) of the bladder retrieved from our Surgical Pathology files from 1998 to 2014. Eight cases were consults. The mean age at presentation was 65 years (range, 39 to 85 y). The M:F ratio was 8:1. The clinical presentation was hematuria and bladder mass in all cases. Six patients had a history of radiotherapy to the pelvis, 5 to treat prostate cancer and 1 to treat uterine cervical cancer. The time from radiotherapy to the diagnosis of EA ranged from 6 to 15 years. The average size of the tumor was 4 cm. (range, 1 to 8 cm.). The submitting diagnoses were poorly differentiated carcinoma (n=5), high-grade invasive urothelial carcinoma (n=3), and atypical vascular proliferation (n=1). Morphologically, the tumors were composed of nests and sheets of highly atypical cells with high nuclear to cytoplasmic ratio, occasional intracytoplasmic lumens, and a hemorrhagic background. None of the cases showed any urothelial carcinoma component. Three patients showed in addition usual angiosarcoma in the resection specimen. By immunohistochemistry, 5/9 cases were positive for cytokeratins, including CK7 (n=3), AE1/AE3 (n=3), and Cam5.2 (n=1). All cases were positive for at least 1 endothelial marker, including CD31 (n=7), CD34 (n=2), FVIII (n=3), and ERG (n=2). Urothelial markers (p63 and GATA3) were consistently negative. Surgical treatment included transurethral resection of the bladder (TURB) only (n=5), TURB followed by cystoprostatectomy (n=2), TURB followed by partial cystectomy (n=1), and cystoprostatectomy only (n=1). The tumor involved the muscularis propria in 5/9 patients, the periureteric adipose tissue in 1 patient, and the prostate and seminal vesicles in 1 patient. Five of 9 patients died of disease, with a median survival of 7 months (range, 6 to 14 mo). Two patients were alive with disease at 3 and 6 months of follow-up. One patient who underwent radical cystoprostatectomy was alive with no evidence of disease 12 months after surgery. EA of the bladder is a rare malignancy that is frequently misdiagnosed as high-grade carcinoma, especially due to positive immunostaining for cytokeratins. This tumor is more frequent in older men with a history of radiotherapy to the pelvis. Morphologic features that should suggest the vascular origin of the tumor include highly atypical nuclei with interspersed erythrocytes, hemorrhagic background, and occasional intracytoplasmic lumens. Patients usually present with muscle invasive disease, and the prognosis is dismal.

Post-radiation atypical vascular proliferation on the head of a young woman: a diagnostic challenge

With improved outcomes associated with radiotherapy (RT), post-irradiation tumors are increasingly seen in long-term cancer survivors. We report a case of a young woman who presented with a three-year history of a vascular lesion on the temple, previously irradiated for a childhood brain tumor. The history of radiation, the clinical appearance, and the biopsy findings of an atypical vascular proliferation in the dermis, were worrisome for a malignant vascular neoplasm and prompted surgical excision. However, further tissue analysis of the excised specimen confirmed a benign atypical vascular lesion (AVL) overlying a banal pilar cyst. Distinguishing post-radiation benign from malignant vascular lesions can be difficult because they share overlapping clinical and histopathologic features. Thus, any vascular lesion that occurs in a previously irradiated field should be excised completely with tumor-free margins and examined histologically.

Androgen Receptor Expression in Vascular Neoplasms of the Breast

Androgen receptors (AR) have been reported to be present in normal breast tissue and breast carcinomas. The following study was undertaken to assess the expression of AR in vascular neoplasms of the breast. All patients with histologically diagnosed hemangioma, angiolipoma, atypical vascular proliferation, and angiosarcoma of the breast were retrieved from the clinical and pathology database at Rush University Medical Center. The slides stained with hematoxylin and eosin were reviewed and immunohistochemical staining for AR (Dako; clone AR441) was performed on paraffin-embedded tissue. Any amount of nuclear staining was considered positive and the intensity of staining was graded as 1+ to 3+. An estimate of the percentage of tumor and stromal cells staining was made. There were a total of 36 cases, 10 hemangiomas, 20 angiolipomas, 2 atypical vascular proliferation, and 4 angiosarcomas. The male to female ratio was 1:4.1. The average age at presentation for men was 45 years and for women was 56.9 years. Anti-AR expression was present in stromal and adipocyte nuclei of the angiolipomas and stromal cells of hemangiomas and angiosarcomas. Interestingly, normal duct epithelium of the breast was positive in 7 of the 29 women and none in men. Androgen expression was present in 62.5% of the hemangiomas, 55% of the angiolipomas, and 50% of the angiosarcomas. The majority of tumors showed a low-intensity nuclear expression of androgens, with 1+ intensity in 13 cases and 2+ intensity seen in 2 cases, and only 1 case of angiolipoma showed 3+ expression. All positive cases of angiolipoma (55%) showed AR in adipocytes and stromal cells. Vascular neoplasms are uncommon tumors in breast and majority of them are benign. Androgen expression is present in >50% of benign vascular neoplasms. Stromal cells and adipocytes typically express AR only in angiolipomas, suggesting a role of androgens in pathogenesis and growth of this neoplasm.

Lymphangiosarcoma of mons pubis following radiotherapy for carcinoma cervix.

Common post-irradiation vascular tumors are post-radiation angiosarcoma, atypical vascular lesions, lymphangiomatosis, and lymphangiosarcoma. While atypical vascular lesions and lymphangiomatosis behave in a benign fashion, post-radiation angiosarcoma and lymphangiosarcoma have a significantly high mortality. Post-radiation angiosarcoma/lymphangiosarcoma usually occurs after an interval of 5–11 years following irradiation for carcinoma of the breast, ovary, uterus, and cervix [1]. This condition is often difficult to diagnose due to its rarity, relatively bland appearance, difficulty in differentiation from radiation-induced changes in the skin, and close mimickers like atypical vascular proliferation and lymphangiomatosis. Meticulous morphological assessment remains the cornerstone to differentiate between angiosarcoma and lymphangiosarcoma from their benign mimikers like atypical vascular proliferation and lymphangiomatosis. It is difficult to differentiate malignant vascular tumors of blood vessel and lymph vessel origin solely on morphological grounds. However, recently, D2-40, a new selective monoclonal marker of lymphatic endothelium, has gained importance in differentiating angiosarcoma from lymphangiosarcoma [2]. Lymphangiosarcoma of the mons pubis following radiotherapy is an extremely uncommon entity. Recently, we have seen such a case where the patient had received radiotherapy for carcinoma cervix 15 years prior to the present illness. To the best of our knowledge, this is the first case of post-irradiation lymphangiosarcoma of the mons pubis in the English literature, and hence it is being reported for its rarity.

Atypical florid vascular proliferation in appendix: a diagnostic dilemma

Vascular abnormality of the intestine is rare, except angiodysplasia. We report on an unusual case of atypical florid vascular proliferations of the appendix. A 41-year old male presented with melena. Adhesioned blood clots in the appendiceal orifice were observed by colonoscopy. He underwent laparoscopic appendectomy. Microscopically, a tiny exophytic polypoid mass was observed. The mass showed pyogenic granuloma-like features in the superficial portion and infiltrative florid vascular proliferations in the deeper portion. Endothelial cells showed minimal nuclear atypia, and mitotic figures were observed infrequently and showed positivity for CD31 and CD34 and negativity for HHV-8. Differential diagnoses include from benign vascular tumor to angiosarcoma or Kaposi’s sarcoma, but this lesion does not fit the description of any defined vascular entity. We diagnosed atypical florid vascular proliferations and the patient has been well during the five-month postoperative follow-up.Virtual SlideThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1386921325843104

Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological, immunohistochemical and molecular analysis of 66 cases

Postradiation cutaneous vascular lesions after treatment of breast carcinoma comprise a heterogeneous group of benign, atypical, and malignant lesions and are best regarded as points along a morphological spectrum. We analyzed a series of cutaneous angiosarcomas after treatment of breast cancer in comparison with control cases and cases of atypical vascular lesions with special emphasis on the expression and amplification of MYC. The 66 cases were divided into control cases (5), cases in which a slight vascular proliferation was seen after radiotherapy of breast cancer (12), cases of atypical vascular lesions after radiotherapy (16), cases of postradiation cutaneous angiosarcomas (25), and cases of angiosarcomas of skin and soft tissues unrelated to radiotherapy (8). None of the control cases (2 M, 3 F, 20–76 years), of cases showing slight vascular proliferation, dermal fibrosis and inflammation after radiotherapy of breast cancer (12 F, 48–79 years), of cases of atypical vascular lesions after radiotherapy (16 F, 29–81 years), and of cases of angiosarcomas of skin and soft tissues unrelated to radiotherapy (3 M, 5 F, 25–92 years) showed an amplification of MYC by FISH analysis. In striking contrast, in all cases of postradiation cutaneous angiosarcomas (25 F, 46-95 years), MYC amplification was found by FISH analysis in a variable number of counted nuclei. Immunohistochemically, strong positive nuclear staining for MYC and prox-1 was seen in cases of postradiation cutaneous angiosarcoma, whereas control cases and cases of atypical vascular proliferation after radiotherapy were negative for MYC, and stained only focally positive for prox-1 in a number of cases. In conclusion, the presence of MYC amplification represents an important additional diagnostic tool in the distinction of postradiation cutaneous angiosarcomas from atypical vascular lesions after radiotherapy. Immunohistochemical stainings for MYC are useful for mapping of these lesions and for careful tumor margin control.