Hemangiopericitoma en el cuello / Hemangiopericytoma in the Neck

Introducción: El hemangiopericitoma es un raro tumor mesenquimal (vascularizado y potencialmente maligno) derivado de los pericitos, que puede aparecer en cualquier parte del cuerpo; sin embargo, en el cuello se describen casos aislados. La resección quirúrgica completa constituye la piedra angular del tratamiento. Objetivo: Presentar un caso de un hemangiopericitoma en el cuello, como un caso inusual, con potencial maligno desconocido, diagnóstico y tratamiento oportuno. Presentación de caso: Paciente de sexo masculino, de 39 años de edad, sin antecedentes de enfermedad conocidos, con una masa perceptible a nivel V del cuello derecho. Estudios de imagen muestran un tumor vascularizado de aproximadamente 6 x 7 x 6 cm, entre los músculos escalenos, que fue originado en la arteria cervical profunda. Se confirmó mediante biopsia incisional el hemangiopericitoma, el cual fue tratado mediante resección tumoral completa y radioterapia adyuvante. Actualmente el paciente no tiene actividad tumoral después de su tratamiento inicial. Conclusiones: El hemangiopericitoma en el cuello es raro, el diagnóstico constituye un reto clínico e histológico, ya que, al ser poco común, su potencial maligno resulta desconocido. Aquellos tumores que tienen bajo grado de malignidad pueden ser controlados, de acuerdo a su localización y tamaño, mediante resección completa; mientras que los tumores de alto grado pueden recurrir y dar origen a metástasis. Nuestro paciente tuvo características histopatológicas con invasión capsular, lo que trajo como consecuencia un incremento del riesgo de recurrencia local. Por ese motivo, se decidió aplicar tratamiento adyuvante con radioterapia. El paciente se mantiene sin recurrencia tumoral local y a distancia después de 9 años de vigilancia médica(AU)

Introduction: Hemangiopericytoma is a rare mesenchymal tumor (vascularized and potentially malignant) derived from pericytes. It can occur anywhere in the body; however, isolated cases are described in the neck. Complete surgical resection is the cornerstone of treatment. Objective: To present a case of hemangiopericytoma in the neck, as an unusual case, with unknown malignant potential, as well as its timely diagnosis and treatment. Case presentation: A 39-year-old male patient, with no known history of disease, had a noticeable mass at the V level of the right neck. Imaging studies showed a vascularized tumor of approximately 6 x 7 x 6 cm, between the scalene muscles, which originated in the deep cervical artery. Hemangiopericytoma was confirmed by incisional biopsy, as well as treated by complete tumor resection and with adjuvant radiotherapy. Currently, the patient has no tumor activity after his initial treatment. Conclusions: Hemangiopericytoma in the neck is rare. Its diagnosis is a clinical and histologic challenge because, being uncommon, its malignant potential is unknown. Those tumors with low-grade malignancy can be controlled, according to their location and size, by complete resection; while high-grade tumors may recur and give rise to metastases. Our patient had histopathologic features with capsular invasion, which resulted in an increased risk of local recurrence. For this reason, adjuvant treatment with radiotherapy was decided to be applied. The patient remains without local or distant tumor recurrence after 9 years of medical surveillance(AU)

Solitary fibrous tumor/hemangiopericytoma treated with temozolomide plus bevacizumab: a report of four cases and literature review.

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare tumor derived from mesenchymal tissue. Although standard chemotherapy for SHT/HPC has not been established, temozolomide plus bevacizumab (TMZ+Bev) therapy for SFT/HPC has been reported. The effectiveness and safety of TMZ+Bev (temozolomide 150 mg/m2 orally on days 1-7 and days 15-21 and bevacizumab 5 mg/kg intravenously on day 8 and day 22 on a 28-day cycle), which was administered from December 2013 until April 2019 to four patients with SFT/HPC, were retrospectively analyzed. Four patients with SFT/HPC received TMZ+Bev. The age of the patients ranged from 41 to 75 years. Two were male, and the primary tumor sites were the meninges in three patients and the pleura in one. One achieved partial response; the others, stable disease (SD). The progression-free survival time ranged from 9.4 to 29.6 months according to RECIST v1.1. One patient died 59 months after using TMZ+Bev, and the others survived for 17 to 64 months. All patients experienced Grade 3 or higher lymphopenia, and three had Grade 3 or higher leukopenia and neutropenia. One patient subsequently received doxorubicin; another, pazopanib. TMZ+Bev therapy for SFT/HPC is safe and effective for maintaining long-term SD.

[Urinary tract hemangiopericytoma treated with radical surgery and neoadjuvant chemotherapy. Report of two cases.] / Hemangiopericitoma en la via urinaria tratados con cirugía radical y quimioterapia neoadyuvante. A propósito de dos casos.

OBJECTIVE: Hemangiopericytoma is a tumor of vascular origin. It is very rare in the prostate. They are generally aggressive tumors. Currently, the treatment of choice is radical surgery. In the literature, there are less than 50 cases described, and neoadjuvant treatment has not been reported in any article. This treatment presents positive responses in another type of sarcomas. Our goal is to report two cases of malignant hemangiopericytoma. The first case was treated with radical surgery and the second case was treated with neoadjuvant chemotherapy before surgery. METHODS: The first case is a 40 year old male. Obstructive urinary symptoms were his first symptoms. A prostate mass with tumor characteristics was seen on ultrasound. After transrectal biopsy, he was diagnosed with hemangiopericitoma. Because the tumor size, neoadjuvant chemotherapeutic prior to radical surgery was decided. The second case is a 77 year old male with an incidental diagnosis of renal mass. After radical nephrectomy, he was diagnosed with hemangiopericitoma. He did not receive adjuvant chemotherapy. RESULTS: In the first case, after neoadjuvant therapy, tumor size was reduced significantly. A pelvic exenteration with radical cystoprostatectomy and rectal resection and Bricker type urinary diversion and colostomy were carried out. In the second case controls after radical nephrectomy were correct and he did not need any treatment. CONCLUSIONS: In urinary tumors, the prostate hemangiopericytoma is a rare entity. Currently, neoadjuvant chemotherapy is not established as treatment for these tumors. For other sarcomas neoadyuvant treatment has good response. In our case, a good result was obtained with neoadjuvant chemotheraphy before surgery. However, a greater number of cases are necessary to establish the use of neoadjuvant chemotherapy in urinary hemangiopericytomas.

OBJETIVO: El hemangiopericitoma es un tumor de origen vascular muy infrecuente en la próstata. Son tumores de características agresivas y actualmente el tratamiento de elección es la cirugía radical. En la literatura existen descritos menos de 50 casos de hemangiopericitomas, y ninguno con tratamiento neoadyuvante. Dicho tratamiento neoadyuvante está descrito en otro tipo de sarcomas con buenas respuestas. Presentamos dos casos de un hemangiopericitoma maligno, uno tratado sólo con cirugía radical y otro con neoadyuvancia quimioterápica previa a la cirugía radical.MATERIAL Y MÉTODOS: El primer caso es un varón de 40 años con síntomas urinarios obstructivos. Se diagnosticó de hemangiopericitoma tras realización de biopsia transrectal. Se decidió neoadyuvancia quimioterápica previa a la cirugía. El segundo paciente es un varón de 74 años con hallazgo accidental de masa renal. Tras nefrectomía radical se diagnosticó de hemangiopericitoma. RESULTADOS: Tras la neoadyuvancia, en el primer paciente, se realizó una cistoprostatectomía radical y una resección de recto con derivación urinaria tipo Bricker y colostomía. El segundo paciente no precisó tratamiento adyuvante tras la cirugía radical. CONCLUSIONES: El hemangiopericitoma es una entidad rara en los tumores urinarios. Actualmente la neoadyuvancia quimioterápica no está establecida como pilar del tratamiento de estos tumores, aunque en otros campos donde los sarcomas son más frecuentes, si que se objetiva mejor respuesta. En nuestro caso obtuvimos una buena respuesta con dicha neoadyuvancia previa a la cirugía, aunque es necesario un mayor seguimiento a una mayor cohorte de pacientes para establecer el uso de la quimioterapia neoadyuvante en los hemangiopericitomas urinarios.

Hemangiopericitoma en la via urinaria tratados con cirugía radical y quimioterapia neoadyuvante. A propósito de dos casos / Urinary tract hemangiopericytoma treated with radical surgery and neoadjuvant chemotherapy. Report of two cases

Objetivo: El hemangiopericitoma es un tumor de origen vascular muy infrecuente en la próstata. Son tumores de características agresivas y actualmente el tratamiento de elección es la cirugía radical. En la literatura existen descritos menos de 50 casos de hemangiopericitomas, y ninguno con tratamiento neoadyuvante. Dicho tratamiento neoadyuvante está descrito en otro tipo de sarcomas con buenas respuestas. Presentamos dos casos de un hemangiopericitoma maligno, uno tratado sólo con cirugía radical y otro con neoadyuvancia quimioterápica previa a la cirugía radical. Material y métodos: El primer caso es un varón de 40 años con síntomas urinarios obstructivos. Se diagnosticó de hemangiopericitoma tras realización de biopsia transrectal. Se decidió neoadyuvancia quimioterápica previa a la cirugía. El segundo paciente es un varón de 74 años con hallazgo accidental de masa renal. Tras nefrectomía radical se diagnosticó de hemangiopericitoma. Resultados: Tras la neoadyuvancia, en el primer paciente, se realizó una cistoprostatectomía radical y una resección de recto con derivación urinaria tipo Bricker y colostomía. El segundo paciente no precisó tratamiento adyuvante tras la cirugía radical. Conclusiones: El hemangiopericitoma es una entidad rara en los tumores urinarios. Actualmente la neoadyuvancia quimioterápica no está establecida como pilar del tratamiento de estos tumores, aunque en otros campos donde los sarcomas son más frecuentes, si que se objetiva mejor respuesta. En nuestro caso obtuvimos una buena respuesta con dicha neoadyuvancia previa a la cirugía, aunque es necesario un mayor seguimiento a una mayor cohorte de pacientes para establecer el uso de la quimioterapia neoadyuvante en los hemangiopericitomas urinarios

Objective: Hemangiopericytoma is a tumor of vascular origin. It is very rare in the prostate. They are generally aggressive tumors. Currently, the treatment ofchoice is radical surgery. In the literature, there are less than 50 cases described, and neoadjuvant treatment has not been reported in any article. This treatment presents positive responses in another type of sarcomas. Our goal is to report two cases of malignant hemangiopericytoma. The first case was treated with radical surgery and the second case was treated with neoadjuvant chemotherapy before surgery. Methods: The first case is a 40 year old male. Obstructive urinary symptoms were his first symptoms. A prostate mass with tumor characteristics was seen on ultrasound. After transrectal biopsy, he was diagnosed with hemangiopericitoma. Because the tumor size, neoadjuvant chemotherapeutic prior to radical surgery was decided. The second case is a 77 year old male with an incidental diagnosis of renal mass. After radical nephrectomy, he was diagnosed with hemangiopericitoma. He did not receive adjuvant chemotherapy. Results: In the first case, after neoadjuvant therapy, tumor size was reduced significantly. A pelvic exenteration with radical cystoprostatectomy and rectal resection and Bricker type urinary diversion and colostomy were carried out. In the second case controls after radical nephrectomy were correct and he did not need any treatment. Conclusions: In urinary tumors, the prostate hemangiopericytoma is a rare entity. Currently, neoadjuvant chemotherapy is not established as treatment for these tumors. For other sarcomas neoadyuvant treatment has good response. In our case, a good result was obtained with neoadjuvant chemotheraphy before surgery. However, a greater number of cases are necessary to establish the use of neoadjuvant chemotherapy in urinary hemangiopericytomas

Transnasal devascularisation of a sinonasal hypervascular tumour (glomangiopericytoma) with direct injection of liquid polymer agent (Squid®).

The technique of direct intratumoral injection of permanent liquid agents has evolved significantly over the past few decades. The percutaneous direct puncture technique is being utilised more and more for embolisation of head and neck hypervascular lesions. We describe a case treated via the direct percutaneous transnasal (TN) puncture technique for embolisation of a sinonasal hypervascular tumour, performed in a 21-year-old male patient. Due to extensive vascularisation of the tumour, preoperative embolisation was requested by our otolaryngologist team in order to minimise intraoperative bleeding and reduce surgical morbidity. A primary attempt at trans-arterial embolisation through selective ophthalmic artery catheterisation was unsuccessful due to unfavourable anatomy. An extensive devascularisation of the nasal fossa lesion was obtained using a single injection of low-density polymeric embolic agent (SQUID®). Post-operative eradication was uneventful. Final histopathology exam revealed vascular tumour consistent with a rare sinonasal glomangiopericytoma. To our knowledge, this is the first reported case of a rare sinonasal glomangiopericytoma treated by direct TN injection, utilising the liquid polymer agent SQUID®.

Efficacy of bevacizumab and temozolomide therapy in locally advanced, recurrent, and metastatic malignant solitary fibrous tumour: A population-based analysis.

BACKGROUND: Patients with locally advanced, recurrent or metastatic solitary fibrous tumour are often treated with bevacizumab and temozolomide based on the clinical efficacy reported in a case series of 14 patients. Given the rarity of solitary fibrous tumour, large trials are not feasible. We report the efficacy of this regimen based on a population-based analysis. METHODS: This was a population-based retrospective, multi-centre analysis using patient data from a provincial cancer registry and treatment database. Cases from June 2006 through October 2016 were identified for patients receiving bevacizumab and temozolomide for locally advanced, recurrent or metastatic solitary fibrous tumour or hemangiopericytoma, which is sometimes used to describe tumours arising from the meninges. The primary outcome was overall response rate. Secondary outcomes included time to response, progression free survival and overall survival estimated using the Kaplan-Meier method. RESULTS: Fourteen patients were identified: median age 59 (range 44-70), male 78.6%. Diagnoses were solitary fibrous tumour in 10 (71.4%) and hemangiopericytoma in four (28.6%), with metastatic disease in 10 (72.7%) patients. The most common primary sites were meninges in four (28.6%) and pelvis in three (21.4%) patients. The median follow-up was 15.5 months, with median treatment of four months. Overall response rate was 21.4% (no complete response, 3 partial response), with median time to response of four months. Median progression free survival, six-month progression free survival and overall survival were 17 months, 65.0%, and 45 months, respectively. CONCLUSIONS: Efficacy of bevacizumab and temozolomide in solitary fibrous tumour appeared to be similar to that previously reported. Our findings confirmed that bevacizumab and temozolomide is an effective and tolerated treatment for this patient population.

Pazopanib efficacy in recurrent central nervous system hemangiopericytomas.

INTRODUCTION: There is currently no treatment for solitary fibrous tumors/hemangiopericytomas (SFT/H) of the central nervous system recurring after multiple surgeries and radiotherapies. The NAB2-STAT6 gene fusion is the hallmark of these tumors, and upregulates Early Growth Factor, activating several growth pathways. METHODS: We treated two patients presenting pluri-recurrent meningeal SFT/H with Pazopanib, a broad-spectrum tyrosine kinase inhibitor. We analyzed the exome and RNA sequencing data of one of them and, in addition to another meningeal SFT/H, compared it to the transcriptomic profiling of 5 systemic SFT/H. RESULTS: A dramatic clinical and radiological response was observed in both cases, respectively 84 and 43% decrease after 3 months. As a comparison, Pazopanib has only a stabilizing effect in systemic SFT/H. Indeed, central nervous system SFT/H show overexpression of different tyrosine kinases targeted by Pazopanib. CONCLUSIONS: Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.

Multifocal Congenital Hemangiopericytoma.

Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported.

A mitotic cause of Whipple's triad: non-islet cell tumour hypoglycaemia in incurable low-grade malignancy.

A 74-year-old man with known metastatic haemangiopericytoma presented with neuroglycopaenic symptoms of confusion and light-headedness secondary to recurrent episodes of fasting hypoglycaemia, which resolved with oral carbohydrate intake. Investigations for hypoglycaemia revealed it to be non-insulin mediated, and subsequently due to a rare paraneoplastic phenomenon termed as non-islet cell tumour hypoglycaemia caused by tumoural overproduction of high molecular weight insulin-like growth factor-II. Despite his underlying malignancy being incurable, directed multimodality treatment involving regular oral carbohydrate intake, glucocorticoid therapy and recombinant human growth hormone was effective in relieving symptoms of hypoglycaemia. We discuss the importance of a systematic diagnostic approach to hypoglycaemia fulfilling Whipple's triad, as specific therapies can be invaluable to improving quality of life.

Hemangiopericytoma of the spleen.

INTRODUCTION: Hemangiopericytoma of the spleen is a very rare tumor, with 14 isolated reports. It was our aim to review our experience and compare it with all the reported cases in an attempt to standardize surgical treatment, adjuvant treatment and follow-up protocol of this infrequent condition. METHODS: A consecutive case series study, with a mean follow-up of 44 months. Five patients (mean age, 49 years) underwent simple splenectomy for hemangiopericytoma limited to the spleen followed by adriamycin-based chemotherapy in one patient. RESULTS: All the patients are alive and free from disease. CONCLUSIONS: For tumors confined to the spleen, simple splenectomy can be considered curative, without any need for further adjuvant treatment. On review of the medical literature, cure can still be achieved with complete resection of recurrences, when feasible, with adjuvant chemotherapy being also indicated. The slow-growing pattern of the tumor suggests a 10-year follow-up.

Collision tumors of the paranasal region: presentation of two cases.

Collision tumors in the paranasal region are extremely rare with limited literature data. To the best of our knowledge, this is the first report of associations of squamous cell carcinoma-esthesioneuroblastoma and lymphoma-hemangiopericytoma in the paranasal region. Preoperatively, radiological and clinical findings should be evaluated carefully for the diagnosis and two or more biopsy specimens should be taken from different morphological parts of the lesions. Adjuvant therapy should be planned according to two different histologies and special importance should be given to the tumor which indicates the prognosis of the patient. A multidisciplinary approach is required for the management of synchronous malignancies.

[Medical treatment of soft tissue sarcomas based on the histological subtype]. / Lágyrészsarcomák gyógyszeres kezelési lehetõségei a szövettani altípus alapján.

The medical treatment of adult soft tissue sarcomas is more and more dictated by the histological subtype, this applies to both cytotoxics and target therapies. Doxorubicin and ifosfamid are the two drugs used either in monotherapy or combination with the best established response rates in adult soft tissue sarcomas for several years. In addition to these compounds there is evidence of efficacy of new drugs such as taxanes in angiosarcoma, gemcitabine+taxanes combination in leiomyosarcomas, trabectedin in leiomyosarcomas and liposarcomas with an extremely high activity in myxoid liposarcoma. With regard to target therapy pazopanib seems especially active in leiomyosarcomas and synoviosarcomas, but totally inactive in liposarcomas, sunitinib and cediranib in alveolar soft part sarcomas, sunitinib and bevacizumab+temozolamide combination in solitary fibrous tumors, and sorafenib in angiosarcomas. mTOR inhibitors are active in PEComas (perivascular epitheloid cell tumors) and crizotinib in ALK rearranged inflammatory myofibroblastic tumors. The efficacy of imatinib and sunitinib in GIST tumors are established, and that of imatinib in dermatofibrosarcoma as well.

Giant mediastinal haemangiopericytoma: an uncommon case.

Haemangiopericytoma is a rare soft tissue tumour characterised by tightly packed tumour cells situated around thin walled endothelial lined vascular channels, ranging from capillary sized vessels to large gaping sinusoidal spaces. The tumour cells are surrounded by reticulin and are negative for muscle, nerve and epithelial markers. The diagnosis of extra-pulmonary intra-thoracic, extra-pleural mediastinal mass is difficult. It constitutes only 6% of all primary tumours and cysts of the mediastinum. We report the rare occurrence of primary intra-thoracic, extra-pulmonary mediastinal haemangiopericytoma of mesenchymal origin with perivascular localisation. The patient underwent right postero-lateral thoracotomy and post-operatively received chemotherapy with adriamycin (60 mg/m2) on day 1 and ifosfamide (1.5 g/m2) on day 1 to 3. Thirty-seven months after the operation, the patient has been well with evidence of a single recurrence in the left lower lobe.

Unusual cause of severe anaemia in a patient with metastatic haemangiopericytoma.

Haemangiopericytoma is a rare tumor of vascular origin. We report the case of patient with severe refractory anaemia due to peripheral destruction of red blood cells by spleen metastases. Anaemia was successfully treated by splenectomy. Afterwards, our patient developed liver and lung metastases and was treated, in a clinical trial, with gefitinib that stabilised the disease during nine years. These interesting features are discussed.

Follicular acneiform eruption induced by bevacizumab.

Bevacizumab is a recombinant humanized monoclonal IgG1 antibody that binds to and inhibits the biologic activity of human vascular endothelial growth factor (VEGF). This antibody, in combination with other antineoplastic agents, is currently used to treat various neoplasms, including colorectal, lung, breast, kidney cancer, and glioblastoma. It is also being used as an off-label intravitreal agent in the treatment of proliferative (neovascular) eye diseases. We report the development of a skin rash with two different patterns in a patient with a hemangiopericytoma of the meninges, a rare aggressive sarcoma, who was treated with repeated intravenous injections of bevacizumab.

Infantile hemangiopericytoma of the orbit treated with primary chemotherapy.

A 7-month-old female infant presented with proptosis of the left eye. Imaging showed an intraconal mass with marked post-contrast enhancement. Histopathology revealed a diagnosis of infantile hemangiopericytoma. The patient was treated with primary chemotherapy with no recurrence until 14 months after completion of treatment.

Sellar and parasellar intravascular lymphoma mimicking pituitary apoplexy.

BACKGROUND: Intravascular lymphoma (IVL) is a rare subtype of large-cell non-Hodgkin lymphoma, characterized by proliferation of lymphoma cells within the lumina of small vessels. There are no previously reported cases of IVL involving the pituitary gland presenting with neuro-ophthalmic findings. METHODS: A 68-year-old female presented with headache, right third nerve palsy, and Horner syndrome. MRI showed a 1.4-cm sellar mass consistent with a pituitary macroadenoma. Two weeks later, despite treatment with dexamethasone, the patient developed complete bilateral ophthalmoplegia and ptosis. Repeat MRI showed invasion of the clivus and cavernous sinuses, and a transsphenoidal pituitary biopsy was undertaken. RESULTS: The preliminary histopathology was consistent with bland pituitary apoplexy, but subsequent examination of an incidentally biopsied nasal polyp revealed endovascular malignant lymphoid cells that, on further scrutiny, were also present in the pituitary tissue. The diagnosis of IVL was confirmed, and the patient had an excellent clinical and radiological response to cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (CHOP-R) chemotherapy. CONCLUSION: IVL may involve the pituitary gland, causing sellar mass effect, cavernous sinus infiltration, and pituitary ischemia, mimicking pituitary apoplexy with neuro-ophthalmic features. It can be effectively treated with CHOP-R chemotherapy.

[Orbital tumor--hemangiopericytoma--in a neonate]. / Guz oczodolu--haemangiopericytoma--u noworodka.

PURPOSE: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology. MATERIAL AND METHODS: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa. RESULTS: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up. CONCLUSIONS: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Hemangiopericitoma: presentación de un caso / Hemangiopericitoma: case presentation

Fundamento: el hemangiopericitoma es un tumor de partes blandas derivado de los pericitos de Zimmerman, células con capacidad contráctil dispuestas en espiral rodeando los vasos sanguíneos. Es un tumor maligno poco frecuente, más propio de adultos entre 50 y 60 años, aunque puede verse en cualquier edad. Existen varios casos reportados en la literatura científica internacional ya que posee varias localizaciones anatómicas. El presente caso es el primero diagnosticado en el Departamento de Anatomía Patológica del Hospital General "Camilo Cienfuegos" de Sancti Spíritus. Presentación de caso: se presenta un caso de una paciente femenina de 62 años, con molestia en hipogastrio la cual fue evaluada clínica y ultrasonográficamente y se le diagnosticó un tumor de ovario, se realizó una cirugía extirpándosele un tumor de localización mesentérica. Se culminó la etapa diagnóstica con una biopsia por parafina a la que se le realizó un examen por microscopía óptica con técnica de hematoxilina y eosina revelando un hemangiopericitoma mesentérico. Conclusiones: el caso reportado tuvo un resultado terapéutico satisfactorio a la combinación de los tratamientos quirúrgico, médico y quimioterapia; demostrando que la combinación de estas tres opciones suele ser útil para lograr una remisión total de la enfermedad previniendo sus recidivas(AU)

Background: Hemangiopericitoma is a tumor in soft tissues derived from the pericites of Zimmerman; the cells have a contractible capacity disposed in spiral surrounding the blood vessels. It is an infrequent malignant tumor, more frequent in adults between 50-60 years old, though it may be seen at any age. There are different cases reported in the internacional scientific literature because it has several anatomic locations. The current case is the first case reported in the Department of Anatomical Pathology of Camilo Cienfuegos General Hospital, Sancti Spiritus city. Case presentation: A 62 year old female patient came to the emergency room complaining of discomfort in the hypogastria. She was clinically and ultrasonographically evaluated and an ovarian tumor was diagnosed. A surgery was performed and a tumor of mesenteric location was removed. The diagnostic stage ended with a biopsy with paraffin wax, and a microscopic study with hemotoxiline and eosin was performed confirming a mesenteric Hemangiopericitoma. Conclusions: the case reported had a satisfactory therapeutic result to the combination of treatments, surgical, medical and chemotherapeutical showing that this combination seems to be useful to manage a total remission of the disease preventing recurrences in this way(AU)

Phase II study of subcutaneous octreotide in adults with recurrent or progressive meningioma and meningeal hemangiopericytoma.

The objective of this phase II study was to evaluate the efficacy and safety of subcutaneous octreotide therapy for the treatment of recurrent meningioma and meningeal hemangiopericytoma. Octreotide is an agonist of somatostatin receptors, which are frequently expressed in meningioma, and reports have suggested that treatment with somatostatin agonists may lead to objective response in meningioma. Patients with recurrent/progressive meningioma or meningeal hemangiopericytoma were eligible for enrollment; those with atypical/anaplastic meningioma or hemangiopericytoma must have experienced disease progression despite radiotherapy or have had a contraindication to radiation. Patients received subcutaneous octreotide with a goal dose of 500 µg 3 times per day, as tolerated. Imaging was performed every 3 months during therapy. The primary outcome measure was radiographic response rate. Eleven patients with meningioma and 1 with meningeal hemangiopericytoma were enrolled during the period 1992-1998. Side effects included diarrhea (grade 1 in 4 patients and grade 2 in 2), nausea or anorexia (grade 1 in 4 patients), and transaminitis (grade 1 in 1 patient). One patient developed extra hepatic cholangiocarcinoma, which was likely unrelated to octreotide therapy. No radiographic responses were observed. Eleven of the 12 patients experienced progression, with a median time to progression of 17 weeks. Two patients experienced long progression-free intervals (30 months and ≥18 years). Eleven patients have died. Median duration of survival was 2.7 years. Immunohistochemical staining of somatostatin receptor Sstr2a expression in a subset of patients did not reveal a correlation between level of expression and length of progression-free survival. Octreotide was well-tolerated but failed to produce objective tumor response, although 2 patients experienced prolonged stability of previously progressive tumors.