Primary Hemangiopericytoma Arising in Extralobar Pulmonary Sequestration: A Coincidence or Two Rare Disorders?

BACKGROUND/AIM: Extralobar pulmonary sequestration (EPS) is an unusual congenital defect characterized by the presence of non-functioning lung tissue receiving arterial supply from the systemic arteries. Primary hemangiopericytoma (HPC) is an uncommon potentially malignant tumor of vascular origin that usually involves the soft tissue of the extremities or retroperitoneum, but extremely rarely affects the lung. We present the rare case of a primary pulmonary HPC arising in an EPS. CASE REPORT: A 65-year-old woman, with dyspnea and pleuritic chest pain, was referred for further investigation. Radiological evaluation demonstrated a well-circumscribed mass above the right hemidiaphragm, receiving its arterial supply from the descending thoracic aorta. The patient underwent a right posterolateral thoracotomy and a middle lobectomy. The intraoperative finding was a well-encapsulated solid mass. The histological evaluation described HPC. RESULTS: The patient remains fit and healthy. CONCLUSION: Pulmonary HPC can arise in EPS. Surgical excision is the treatment of choice.

Sinonasal hemangiopericytoma caused hypophosphatemic osteomalacia: A case report.

RATIONALE: Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic syndrome featured with fibroblast growth factor 23 (FGF23) secretion primarily by benign mesenchymal tumors and sometimes by malignancies. TIO diagnosis and treatment is often delayed because TIO usually has nonspecific generalized bone pain and weakness, and location of TIO tumor is quite challenging. Very few TIO caused by sinonasal hemangiopericytoma have been reported in the literature. PATIENT CONCERNS: A 40-year-old Chinese woman presented with diffuse bone pain for more than 1 year. Laboratory examination showed hypophosphatemia, hyperphosphaturia, hypocalcemia, an elevated serum alkaline phosphatase (ALP) level and bone-specific ALP level. Imaging studies revealed low bone mineral density (BMD) and multiple pseudofractures at the ribs. F-18 fluorodeoxyglucose positron emission tomography was negative in searching for tumors. Because no tumor was located, the patient was treated with oral phosphate, calcium, and alfacalcidol, and achieved great relief in her symptoms and improvement in BMD. Six years later, the patient had breast cancer surgery and received chemotherapy, and still had hypophosphatemia. During this time, nasopharyngo-fiberscope showed nasal mass in her left nasal cavity. Then she had her nasal polyps removed and surprisingly the serum phosphate became normal. DIAGNOSES AND INTERVENTIONS: The patient had the nasal mass resected, and pathological diagnosis of the nasal mass was sinonasal hemangiopericytoma. Immunohistochemical analysis was positive for FGF23. Thus the final diagnosis was osteomalacia induced by sinonasal hemangiopericytoma. Phosphate supplementation and alfacalcidol were discontinued. OUTCOMES: The patient had normal serum phosphate after 6-month follow-up. LESSONS: By presenting this case, we hope to remind clinicians that in patients with osteomalacia with undetermined reason and intranasal polypoid mass, sinonasal hemangiopericytoma should be suspected.

Traumatic Facial Myopericytoma: A Differential to Consider.

Myopericytoma is a slow-growing, benign soft tissue neoplasm that arises from perivascular smooth muscle cells. This tumor is a rare entity itself, but it is only scarcely mentioned in the literature occurring secondary to trauma. The authors report a 21-year-old male patient who presented with a pulsatile mass in the medial canthal area where he had experienced previous trauma from a car accident 1-year prior. The mass was excised and histopathology revealed myopericytoma. This clinical report adds to the limited body of evidence supporting trauma as an etiology for this rare tumor.

Intravascular Myopericytoma of the Plantar Region: Case Report and Discussion of the Probable Origin From a Cutaneous Vascular Malformation.

Myopericytoma is a perivascular myoid neoplasm of skin and soft tissues characterized by numerous thin-walled blood vessels surrounded concentrically by round to spindle myoid tumor cells, which shows α-smooth muscle actin and h-caldesmon coexpression and commonly negativity for desmin. These tumors arise predominantly in extremities of adult patients with benign clinical course. Based on the architectural pattern, there are various histologic variants as classical-solid myopericytoma, hemangiopericytoma-like myopericytoma, angioleiomyoma-like myopericytoma, myofibroma-like myopericytoma, hipocelular fibroma-like myopericytoma, intravascular myopericytoma, cellular immature myopericytoma, and malignant myopericytoma. The authors report a case that fully satisfies the morphological and immunohistochemical criteria for intravascular myopericytoma, which plantar location is not previously described in the literature. In addition, the authors discuss about its possible development from a preexistent cutaneous vascular malformation.

[Chronic dacryocystitis 24 years after dacryocystorhinostomy?]. / Chronische Dakryozystitis 24 Jahre nach Dakryozystorhinostomie? Diagnose: Extraorbital gelegenes Hämangioperizytom (solitärer fibröser Tumor bzw. SFT nach WHO).

A 50-year-old woman suffered from recurrent swelling and inflammation in the region of the medial portion of the lower eyelid 24 years after right-sided dacryocystorhinostomy. The tumor was removed in two stages under histological control of the excision margins. No evidence for malignancy was found. During the follow-up period of 4 years the patient remained recurrence free and without complaints. The diagnosis reached was an extraorbital hemangiopericytoma.

Perivascular myoma of myopericytoma and myofibromatosis-type arising in a chronic scar.

We describe a case of a cutaneous perivascular myoma with features overlapping between the myofibromatosis and the myopericytoma type. The patient is a 58-year-old woman with a painless plaque-like and multinodular lesion in the pretibial dermis and subcutaneous tissue. She had repeated trauma to this site, first in her early youth that left an area of hyperpigmentation, and then again at age 40. The biopsy showed a biphasic pattern with a myofibromatosis-type component composed of spindle cell myoid nodules and more cellular round cell areas. The myopericytoma-like areas appeared to be infiltrating along vessels. These areas contained aggregates of immature-appearing cells arranged concentrically around vascular lumina in a manner reminiscent of pericytes. Immunohistochemical stains showed focal positivity for smooth muscle actin. Immunohistochemical and ultrastructural studies have showed these pericyte-like cells to be of a myoid origin. The reason for the neoplastic proliferation of perivascular myoid cells is presently unknown. The association of trauma and neoplastic transformation of the skin is rare. We report the first case of a cutaneous perivascular myoma arising in a chronic scar.

[A case of haemangiopericytoma of the spermatic cord].

A case of haemangiopericytoma of right spermatic cord is reported. A 50-year old male presented with a month-lasting painless swelling of right scrotum. Ultrasonography revealed a soft tissue mass in right spermatic cord, which was about 3.5 cm in diameter. Right high inguinal orchiectomy was performed. The tumor was solid, smooth surfaced and well circumscribed. Histologically, the tumor had many capillary vessels and short spindle calls around the vessels. On the silver impregnation, argyrophil fibers surrounded the tumor cells. Immunohistologically, tumor cells were positive for CD34 antigen and negative for factor VIII antigen. The mitotic rate was 2 per highpower field. Accordingly, this tumor was diagnosed as benign haemangiopericytoma. The patient is doing well without any sign of recurrence, as of 30 months post-operatively. Haemangiopericytoma is a rare neoplasm of pericyte origin. It commonly occurs in retroperitoneum and lower extremities. To our knowledge, only two cases of malignant haemangiopericytoma of the spermatic cord was reported, and this case is the first benign case of the spermatic cord.

Ten-year-old boy with hemangiopericytoma and human immunodeficiency virus infection.

This report documents the first case of hemangiopericytoma in an HIV-infected child who is most likely a case of vertical transmission of HIV with slow progression to AIDS. We also raise the possibility that there is a causal relationship between HIV and hemangiopericytoma.

Exposure to polymeric materials in vascular soft-tissue sarcomas.

UNLABELLED: Known etiologic factors related to endothelial angiosarcomas are exposures to arsenic, thorium dioxide, therapeutic irradiation, and certain congenital diseases. Little is known on the etiology of hemangiopericytomas. Since 1974, several reports have appeared on a distinct relationship between the exposure to vinyl chloride monomers and angiosarcomas of the liver. The early reports on this matter provided the reason to collect the occupational histories of vascular sarcomas accumulated since that time. METHODS: Data on the occupational histories of patients with different forms of angiosarcomas, treated between 1975 and 1995 in two institutions, were prospectively collected and analyzed. In this personal series the only selection criteria were the referral of patients for postoperative or palliative irradiation and their personal care by the author. FINDINGS: Among 21 adult cases of vascular sarcomas there were 4 patients with occupational exposure to vinyl chloride (VC) either alone or together with other artificial polymers. Seven other patients showed exposure to several plastics or resins other than VC. Altogether, 11 of 21 (52%) of the explored patients were found to have been exposed to artificial polymeric materials over a mean period of 18 years. The patients without such exposure were 4 farmers, 2 house-wives, and 1 woodworker, telephonist, mason, and inland revenue official, respectively. Two cases were radiation-induced. The series contained no angiosarcoma of the liver. INTERPRETATION: This study offers new evidence of the occurrence of vinyl-chloride-induced angiosarcomas outside the liver and confirms observations that have previously been published in case reports. Moreover, it may be suspected from this analysis that polyvinyl chloride and its monomers are not the only polymeric materials that may contribute to an induction of angiosarcomas in humans. Repeated occupational histories have to be taken from the patients to achieve data of the greatest value, since there are many professional activities that do not primarily lead to the assumption of specific exposure to polymeric materials.

Hemangiopericytoma arising at the site of a dural graft 13 years after total removal of a benign meningioma--case report.

A 64-year-old female presented with hemangiopericytoma arising from a dural fascia graft placed after a previous operation for the total removal of a benign meningioma. The patient also had multiple pulmonary metastases identified as hemangiopericytoma. The tumor resected at the first operation was benign based on all grading systems for meningiomas. We considered that the tumor was a kind of scar tumor. The hemangiopericytoma probably developed from a scar tumor at the graft. We emphasize the importance of long-term follow-up for patients who have undergone surgery for benign tumors.

[A case of hemangiopericytoma in the pelvic retroperitoneum].

A case of hemangiopericytoma in the pelvic retroperitoneal space is described. A 54-year-old-male had a retroperitoneal mass which had been incidentally detected during evaluation of microscopic hematuria. The pelvic arteriogram revealed an extremely hypervascular mass at the arterial phase and intensive, longlasting and well-demarcated tumor stain at the capillary phase, which were characteristic of hemangiopericytomas. Resection of the tumor was difficult due to a well developed vessel mesh around the tumor and adhesion to the surrounding tissues. The tumor weighted 45 g. The patient has remained free of disease for 29 months postoperatively. Since histopathological malignant features of hemangiopericytoma are still obscure, long term and close follow up is important.

Hemangiopericytoma of the orbit treated with conservative surgery and radiotherapy.

A 23-year-old man had two recurrences following local excision of a hemangiopericytoma of the right orbit. A third local excision was followed by a course of postoperative radiation therapy (64.8 Gy [6480 rad] in 36 treatments). Seven and a half years following therapy, the patient is disease free and has experienced no side effects.

Hemangiopericytoma of the heart following treatment of Hodgkin's disease. A case report.

During the autopsy of a 24 year old woman, who died of cardiorespiratory insufficiency a large solitary tumour was found extending into the right ventricle of the heart and obstructing the pulmonary valve subtotally. Histologically the tumour showed a vascular pattern of differentiation typical of a hemangiopericytoma with almost uniform cellularity and a dense reticulin meshwork surrounding the individual tumour cells. Ten years before death the patient had been diagnosed as having Hodgkin's disease treated with an unusually high dose of radiotherapy combined with chemotherapy. Although tumours have been known to arise following radiation and chemotherapy and hemangiopericytomas of almost all parts of the body have been described, to our knowledge this is the first case of an (iatrogenically induced) hemangiopericytoma of the heart.

[Changes in the biochemical parameters of lymphocyte functional activity in simian adenovirus SA7(C8)-induced carcinogenesis]. / Izmenenie nekotorykh biokhimicheskikh parametrov funktsional'noi aktivnosti limfotsitov pri kantserogeneze, indutsirovannom adenovirusom SA7(C8) obez'ian.

A functional defect of lymphocytes in hamsters with tumours during carcinogenesis induced by monkey adenovirus SA7(C8) was found and estimated by means of the blast transformation reaction. An increased activity of transport ATPases, the disturbance of cyclic nucleotide levels (cAMP, cGMP) and a decrease in both basal and Con A-activated calcium transportation were observed simultaneously with changes in the activation of immunocompetent cells under Con A influence.

[T- and B-cell functional activity in the immune response in viral carcinogenesis]. / Issledovanie funktsional'noi aktivnosti T- i B-kletok v immunnom otvete pri virusnom kantserogeneze.

The functional activity of the spleen cells, their ability to produce antibody-forming cells on the model of the adoptive transfer, activity of T-helper-cells and B-precursor-cells of antibody producers in the tumour growth induced by monkey SA7 (C8) adenovirus were studied. The inoculation of the tumourigenic virus to the newborn mice CBA, inhibit antibody formation in the system of adoptive transfer already in the latent period with a sharp inhibition at the terminal stages of carcinogenesis. The helper activity is suppressed earlier than the activity of B-precursors of antibody producers. The obtained data indicate a considerable immunodepressive action of monkey adenovirus SA7 (C8).

Haemangiopericytoma in otolaryngology.

Haemangiopericytoma is a rare vascular tumour with variable pathological behaviour. It arises from the proliferation of special cells called 'pericytes' which are found outside the blood capillaries. There is no specific predilection for any site in the body. Clinically and histologically, it can be benign or malignant, but this differentiation sometimes becomes rather a hard task for the pathologist. Wide surgical excision is the treatment of choice. Radiotherapy and chemotherapy can be valuable adjuncts. Only fourteen cases of nasal haemangiopericytoma have been reported in the literature and these are individually set out in Table I. Another case is reported in this paper, with a review of all the tumours in the field of Otorhinolaryngology since 1942.

Hemangiopericytoma. An analysis of 106 cases.

A series of 106 cases of hemangiopericytoma was analyzed. The neoplasms occurred principally in adults (median age, 45 years), were deep seated, and were most common in the thigh (27 cases) and the pelvic retroperitoneum (26 cases). A painless mass was the first symptom in 96 of the patients. The median size of the excised tumors was 6.5 cm. Surgical removal of the tumor was often complicated by hemorrhage because of marked dilatation of the vascular bed in the vicinity of the neoplasm, probably as the result of rapid exchange of blood from the arterial to the venous circulation within the tumor. Microscopically, benign and malignant forms could be distinguished. The latter were characterized by increased cellularity, prominent mitotic activity, and foci of necrosis or hemorrhage. Follow-up information was obtained in 93 cases. Seventy-one of the 93 patients were living (two with recurrence and four with metastasis), and 22 had died (13 as the result of recurrence or metastasis in more than two-thirds of the related causes). Recurrence preceded metastasis in more than two-thirds of the patients with evidence of metastasis. The 10 year survival rate was 70 per cent. The morphologic differences from other mesenchymal tumors showing a hemangiopericytoma-like vascular pattern are discussed, and the close resemblance of hemangiopericytoma to richly vascular forms of fibrous histiocytomas and synovial sarcoma is emphasized. Congenital or infantile hemangiopericytoma is described as a separate entity having a distinctive microscopic pattern and behavior.

Blood-vessel neoplasms in children: epidemiologic aspects.

In a search for etiologic leads to blood-vessel neoplasms, we examined 111 death certificates of U.S. children who died from 1960 to 1968 of angiosarcoma, hemangioendothelioma, and hemangiopericytoma and 127 medical records of similar cases from 12 institutes. The available data provided no leads to environmental agents (vinyl chloride, thorotrast, arsenic) that can produce vascular liver tumors in adults, but one infant, who died from a hepatic tumor, lived within a mile of an industrial source of polyvinyl chloride. About half of the children with hepatic hemangioendotheliomas had associated skin hemangiomas, which may aid in the differential diagnosis of liver tumors in infancy. Hepatic hemangioendotheliomas also predominated in girls, a possible clue to the origin of the tumor. A familial influence was suggested by one sibling aggregation of cutaneous hemangioendotheliomas.