scholarly journals Primary alveolar soft tissue sarcoma of the central nervous system. Case report

2021 ◽  
Vol 26 (3) ◽  
Author(s):  
Eliezer Villanueva-Castro ◽  
Marcos Vinicius Sangrador-Deitos ◽  
Jorge Fernando Aragón-Arreola ◽  
Gerardo Cano-Velázquez ◽  
Juan Luis Gómez-Amador ◽  
...  

Background: Alveolar soft part sarcoma (ASPS) is a rare, slow-growing soft tissue tumor with uncertain etiology; it is considered among the least common sarcomas, representing 0.2-1% of these cases in large studies. These tumors usually appear during childhood or young patients, with predominance in females. Case description: We introduce the case of ASPS in a 62-year-old man, who presented with 7 months of progressive headache and diplopia. The brain MRI showed an infiltrative lesion in the anterior fossa that extended to the right orbital roof. The possibility of metastasis was ruled out.  The patient underwent resection of the tumor with posteriorly good visual and neurologic recovery. Histologic characterization demonstrated homogeneous eosinophilic cells with a solid, vascularized pattern, cells with large and binucleated nucleoli, and vessels with endothelial and myoepithelial hyperplasia; numerous apoptotic bodies and mitosis figures were also present, but no necrosis. On immunohistochemistry, cells exhibited positive CD56, NSE in membrane form, and slight myogenin; vessels were strongly positive for myogenin, myoglobin, CD34, CD31, factor VIII, vimentin, and nestin as well as for HBM45, CD20, GFAP, and S-100; cytokeratin showed fine extracellular and intracellular filaments; GATA and TTF1 were negative.  Some clear cells were observed to be positive for CD68. The piece was diagnosed as a non-meningeal alveolar sarcoma of the soft tissue with solid pattern. Discussion and Conclusion: This case corresponds to the second tumor of this kind presented at our institution, the first one reported, and perhaps, one of the oldest patients to develop it worldwide.

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Eduardo Cambruzzi ◽  
Enilde Eloena Guerra ◽  
Hamilton Cardoso Hilgert ◽  
Herbert Jorge Schmitz ◽  
Vinícius Lopes Silva ◽  
...  

Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET) represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.


2014 ◽  
Vol 21 (4) ◽  
pp. 471-476
Author(s):  
Danil Adam ◽  
Toma Papacocea ◽  
Ioana Hornea ◽  
Cristiana Moisescu

Abstract Meningioma is in most cases a benign tumor of the central nervous system with two growth patterns: en masse and en plaque. Hyperostosis is associated in 13 - 49 % of the cases with en plaque meningioma. We describe the case of a 47 years old woman with meningotelial sphenoid ridge meningioma which was totally removed. At the first admission she presented with no neurological deficits, seizures and a mild right exophthalmos. This had an indolent growth. After 10 years, the patient was readmitted for headache, blurred vision and right exophthalmos. Skull X-rays and brain MRI revealed an important thickening of the right superior orbit wall and sphenoid ridge. She underwent a new surgery. There was no intradural tumor found. Instead, bones of the superior and lateral right orbit walls were very hiperostotic. A hole of 3/2 cm in the right superior orbital wall was drilled and the orbital cavity was decompressed. In the postoperative period, the symptoms were remitted and the exophthalmos reduced. We discuss the causes and management of hyperostosis associated with meningiomas.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
R. F. Falkenstern-Ge ◽  
M. Kimmich ◽  
M. Wohlleber ◽  
A. Grabner ◽  
G. Friedel ◽  
...  

A 30-year old woman was referred to our center because of suspicion of a primary lung tumor of the right upper lobe. Histological examination of the lung lesion revealed lung metastasis of a previously treated alveolar soft part sarcoma of the musculus vastus medialis of the right femur, which was resected 20 years ago. Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. It is a slow-growing malignant soft tissue tumor arising in muscle tissue, usually in young adults. Due to pleural and extensive mediastinal infiltration with bilateral lung metastases, a systemic treatment with chemotherapy doxorubicin and ifosfamide was initiated. Late metastases from previously treated alveolar part sarcoma should be considered in patients with suspicious lung lesions even if surgical treatment was performed a long time ago.


2020 ◽  
Vol 13 (1) ◽  
pp. 71-75
Author(s):  
Iliya V. Valkov ◽  
Milan N. Mladenovski

Summary The term meningeal melanocytoma was first introduced by Limas and Tio in 1972 to figure out lesions of the central nervous system that, under light microscope view, have meningioma characteristics, and ultrastructural characteristics of melanocyte neoplasm, respectively. Meningeal melanocytomas (MM) represent 0.06-0.1% of brain tumors. The annual incidence is about 1 per 10 million. This type of neoplasm is rarely seen in clinical practice. A few cases have been reported in the literature. A 62-year-old man was admitted, complaining of trigeminal pain in the area of the I and II branches of CN V on the left of his face. He underwent two surgeries in a row. First, malignant melanoma, and then meningeal melanocytoma were histologically verified. Neurological examination demonstrated hyperpigmented left iris, neuralgic pain in I and II branches of CN V, latent central hemiparesis and hemihypesthesia for the right extremities, positive Babinski reflex on the right, positive axial pathological reflexes, and partial motor aphasia. Primary intracranial meningeal melanocytoma is difficult to diagnose preoperatively because of the tumor’s non-specific clinical and neuroradiological characteristics. So electron microscopy and immunohistochemical additional diagnostic confirmation are mandatory. Immunohistochemical findings - the presence of S-100 protein, vimentin, and antimelanocyte antigen HMB-45, no reaction for EMA and ultrastructural (melanosomes in different maturation, contacts type zonula adherens) are all in favour of the histopathological diagnosis of MM. Meningeal melanocytoma is a slow-growing tumor, biologically benign, and attempts for surgical radicalism are recommended. It is not entirely clear whether a malignant transformation of these tumors is possible. The role of chemotherapy also remains questionable and has not been documented as effective.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Shokouh Taghipour Zahir ◽  
Fateme Salemi

Background. Neuroblastoma is a solid tumor that occurs more frequently in pediatric populations. It may originate from any part of the sympathetic nervous system, but it most commonly arises from the paraspinal sympathetic ganglia in the abdomen or mediastinum. Local lymphadenopathy and distant metastasis to the central nervous system, orbit, and liver might be detected; however, it rarely includes soft tissue or musculoskeletal involvement. Case Report. Here, we report a 10-month-old infant presented with a right thigh mass with an otherwise benign physical exam and medical history. MRI of the lower extremities suggested tumoral infiltration in the soft tissue of both thighs, predominantly on the right side. Surgical pathology of the lesion confirmed neuroblastoma. A large subhepatic mass and paraaortic lymphadenopathy in the abdominal CT scan and metaiodobenzylguanidine scan findings favored primary abdominal neuroblastoma that had spread to lower extremities. The patient has been in remission since the completion of chemotherapy. Conclusion. Neuroblastoma should be considered in infants with an abnormal mass in extremities. Due to its aggressive nature, most patients struggle with distant and local tumor spread at diagnosis. Therefore, any abnormal signs and symptoms, especially in younger pediatrics, warrant immediate evaluation to avoid tumor expansion.


1991 ◽  
Vol 30 (01) ◽  
pp. 35-39 ◽  
Author(s):  
H. S. Durak ◽  
M. Kitapgi ◽  
B. E. Caner ◽  
R. Senekowitsch ◽  
M. T. Ercan

Vitamin K4 was labelled with 99mTc with an efficiency higher than 97%. The compound was stable up to 24 h at room temperature, and its biodistribution in NMRI mice indicated its in vivo stability. Blood radioactivity levels were high over a wide range. 10% of the injected activity remained in blood after 24 h. Excretion was mostly via kidneys. Only the liver and kidneys concentrated appreciable amounts of radioactivity. Testis/soft tissue ratios were 1.4 and 1.57 at 6 and 24 h, respectively. Testis/blood ratios were lower than 1. In vitro studies with mouse blood indicated that 33.9 ±9.6% of the radioactivity was associated with RBCs; it was washed out almost completely with saline. Protein binding was 28.7 ±6.3% as determined by TCA precipitation. Blood clearance of 99mTc-l<4 in normal subjects showed a slow decrease of radioactivity, reaching a plateau after 16 h at 20% of the injected activity. In scintigraphic images in men the testes could be well visualized. The right/left testis ratio was 1.08 ±0.13. Testis/soft tissue and testis/blood activity ratios were highest at 3 h. These ratios were higher than those obtained with pertechnetate at 20 min post injection.99mTc-l<4 appears to be a promising radiopharmaceutical for the scintigraphic visualization of testes.


2021 ◽  
pp. 106689692110219
Author(s):  
John L.S. Cunha ◽  
Marco A. Peñalonzo ◽  
Ciro D. Soares ◽  
Bruno A.B. de Andrade ◽  
Mário J. Romañach ◽  
...  

Oncocytic lipoadenoma (OL) is a rare salivary gland tumor characterized by the presence of oncocytic cells and mature adipose tissue. To date, only 30 cases of OL have been reported in the English-language literature. We present 3 additional OL cases involving the parotid, including a synchronous presentation with paraganglioma of the right carotid bifurcation. Microscopically, both the OLs were composed of a mixed population of oncocytes and adipocytes in varying proportions surrounded by a thin, connective tissue fibrous capsule. Oncocytes were positive for pan-cytokeratins (CKs) AE1/AE3, epithelial membrane antigen, CK5, CK7, CK14, CK18, and CK19. Calponin, p63, alpha-smooth muscle actin, and carcinoembryonic antigen were negative. Vimentin and S-100 protein were positive only in adipose cells. Despite distinctive morphologic features, OL is often misdiagnosed, given its rarity. We hope to contribute to surgeons’ and pathologists’ awareness and knowledge regarding the existence of this tumor and provide adequate management through conservative surgical excision.


2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.


2021 ◽  
pp. 1-5
Author(s):  
Karishma Habbu ◽  
Roshan George ◽  
Miguel Materin

<b><i>Purpose:</i></b> This report describes a case of relapsed primary breast lymphoma (PBL) presenting as vitreoretinal lymphoma (VRL). <b><i>Methods:</i></b> We describe the clinical and hematopathologic findings in a patient with relapsed PBL involving the vitreous of both eyes. <b><i>Results:</i></b> A 59-year-old woman was treated for PBL with systemic and intrathecal chemotherapy 5 years prior to presentation. Three years later, she presented to an outside clinic with blurred vision in both eyes and bilateral vitritis. She was referred to our clinic with concern for ocular lymphoma. On presentation, the patient’s best-corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye with 3+ vitreous cells in the right eye and 2+ vitreous cells in the left eye. Vitreous biopsy of the right eye revealed CD5-negative/CD10-negative B-cell lymphoma cells on flow cytometry. She had no evidence of disease on brain MRI, lumbar puncture, bone marrow biopsy, or full-body CT scans. She was treated with a regimen of rituximab, methotrexate, procarbazine, and vincristine for central nervous system penetration as well as multiple intraocular injections of methotrexate and rituximab with improvement in vision and ocular inflammation bilaterally. <b><i>Conclusion:</i></b> Relapsed PBL can present as bilateral VRL.


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