Suprasellar Melanocytoma with Leptomeningeal Seeding: An Aggressive Clinical Course for a Histologically Benign Tumor

Introduction. Meningeal melanocytoma (MM) is a very rare neuroectodermal neoplasm arising from the leptomeninges. Primary suprasellar melanocytomas are exceedingly rare, with only a handful of cases reported. The systemic spread of a nontransformed meningeal melanocytoma is an unusual occurrence. Herein, we report the first case of a primary sellar melanocytoma with cerebral and spinal meningeal seeding. Case Report. A 30-year-old male with no previous medical history presented to the endocrinology department with a loss of body hair. The endocrine workup concluded with isolated hypogonadotropic hypogonadism. The Magnetic Resonance Imaging (MRI) of the brain and sella revealed a large suprasellar mass continuous with the infundibulum of the pituitary gland. It was heterogeneously hyperintense on T1-, T2-, and FLAIR-weighted images and was enhanced with contrast, along with cerebral and spinal leptomeningeal spread. The patient was referred to the neurosurgery department, and a lumbar spine biopsy was indicated. The histopathological examination was suggestive of a grade I meningeal pigmented melanocytoma. Conclusion. Thus, primary sellar melanocytomas with leptomeningeal spread are an extremely rare phenomenon. Metastatic malignant melanoma should be ruled out. Being aware of differential diagnosis and the unusual behavior of meningeal melanocytoma will be necessary to manage the patient appropriately. Complete tumor resection is the best treatment whenever possible, and radiotherapy should be considered in case of unresectability or partial resection.

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Neuroendocrine tumor arising from the greater omentum treated with laparoscopic tumor resection: a case report

Surgical Case Reports ◽

10.1186/s40792-021-01217-4 ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Taichi Terai ◽

Kenji Nakagawa ◽

Kota Nakamura ◽

Shunsuke Doi ◽

Kohei Morita ◽

...

Keyword(s):

Neuroendocrine Tumor ◽

Histopathological Examination ◽

Tumor Resection ◽

Ventral Side ◽

Greater Omentum ◽

Preoperative Imaging ◽

Ki 67 ◽

Case Presentation ◽

First Case ◽

Omental Tumor

Abstract Background Primary omental tumors are extremely rare. Herein, we report the first case of a primary omental neuroendocrine tumor (NET). Case presentation A 59-year-old woman was referred to our hospital for the treatment of an 18-mm tumor located at the ventral side of the duodenum. No other tumor was detected. The preoperative imaging diagnosis was omental tumor. A laparoscopic tumor resection was performed. Histopathological examination revealed that the tumor consisted of cuboidal cells with eosinophilic, granular cytoplasm showing trabecular or ribbon architecture. No other component was seen. The mitotic count was of 5 per 10 high-power fields. Immunohistochemical staining was positive for chromogranin A, synaptophysin, and CD56. Her Ki-67 index was 5%. These results led to the diagnosis of grade 2 omental NET. The patient was discharged on the 3rd postoperative day without any complications and did not develop any recurrence for 3 years. Conclusions We encountered a very rare case of omental NET. Complete resection is recommended with minimally invasive surgery for the diagnosis of NET.

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NCOG-57. NEUROPSYCHOLOGICAL FUNCTION IN MENINGEAL MELANOCYTOMA: A CASE REPORT

Neuro-Oncology ◽

10.1093/neuonc/noaa215.595 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii142-ii142

Author(s):

Brandon Baughman ◽

Kinsey Pebley ◽

Manjari Pandey ◽

Matthew Ballo ◽

Madison Michael

Keyword(s):

Case Report ◽

Cognitive Processing ◽

Formal Education ◽

Neuropsychological Testing ◽

Pineal Region ◽

Communicating Hydrocephalus ◽

Neuropsychological Function ◽

Meningeal Melanocytoma ◽

Leptomeningeal Spread ◽

First Case

Abstract Meningeal melanocytomas are rare tumors derived from leptomeningeal melanocytes. The aggressive subtype is considered a rare variant of malignant melanoma, carrying a poor prognosis. To date, the extant literature is void of any studies that have systematically assessed cognition or neuropsychological function in these patients. We present a case report of a 61-year-old male, right-hand dominant, Caucasian male with 16 years of formal education. He has an unremarkable medical history, and his presenting symptom were syncopal episodes. An extensive work-up revealed a complex pineal region mass. Serum markers (CEA, AFP, and beta HcG) were negative. The patient continued to deteriorate neurologically and underwent a gross total resection of the mass. At the time of resection, there was evidence of definitive leptomeningeal dissemination. Because of the leptomeningeal spread, he underwent whole brain radiation therapy with a boost to the cerebellum and surgical site. He subsequently required implantation of a ventriculoperitoneal shunt for treatment of communicating hydrocephalus. Prior to WBRT, the patient underwent comprehensive neuropsychological testing. According to the patient and his spouse, they estimated functioning at approximately 50% of normal cognition, with precipitous decline noted prior to resection of the lesion. At the time of the exam, he was dependent in essentially all instrumental activities of daily living. The examination identified a pattern consistent with diffuse subcortical involvement, characterized by impairments in cognitive processing speed, attention, working memory and executive function. Conversely, the patient demonstrated no amnesia or higher cortical dysfunction. Moreover, mood, anxiety, or other psychopathology failed to account for his cognitive impairment. In our review, this is the first case in the literature of a patient with meningeal melanocytoma (with leptomeningeal spread) who has been comprehensively assessed neuropsychologically. Conceptualizations, treatment, and prognosis will be discussed.

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Vertebral synovial chondromatosis

Journal of Neurosurgery Spine ◽

10.3171/spi.2004.1.2.0211 ◽

2004 ◽

Vol 1 (2) ◽

pp. 211-218 ◽

Cited By ~ 22

Author(s):

Gary L. Gallia ◽

Nirit Weiss ◽

James N. Campbell ◽

Edward F. McCarthy ◽

Anthony P. Tufaro ◽

...

Keyword(s):

Posterior Approach ◽

Facet Joint ◽

Histopathological Examination ◽

Tumor Resection ◽

Synovial Chondromatosis ◽

Low Grade ◽

Radiographic Findings ◽

Content Type ◽

First Case ◽

Fine Print

✓ Synovial chondromatosis is an uncommon disorder characterized by the formation of multiple cartilaginous nodules within the synovium, most commonly affecting large joints. Its involvement with the spine is rare; only six cases have been reported. The authors describe two patients with synovial chondromatosis involving the cervical spine. In the first case, synovial chondromatosis arose from the left C1–2 facet joint. This patient underwent a two-stage procedure including a posterior approach for tumor resection and occipitocervical fusion as well as a transmandibular circumglossal approach to the anterior craniocervical junction to complete the tumor removal. Interestingly, on histopathological examination, scattered foci of low-grade chondrosarcoma were intermixed within the synovial chondromatosis. To the authors' knowledge, this is the first report of secondary low-grade chondrosarcoma arising in vertebral synovial chondromatosis. In the second case, synovial chondromatosis involved the left C4–5 facet joint. Tumor resection and cervical fusion were performed via a posterior approach. In this report, the authors describe the clinical presentation, radiographic findings, operative details, histopathological features, and clinicoradiological follow-up data obtained in these two patients and review the literature pertaining to this rare entity.

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Intestinal Heterotopic Pancreas Involved by Simultaneous PanIN-2 Lesion and Endocrine Microadenoma: a Unique Case

Journal of Gastrointestinal and Liver Diseases ◽

10.15403/jgld.2014.1121.262.pan ◽

2017 ◽

Vol 26 (2) ◽

pp. 199-202

Author(s):

Remus Cornea ◽

Sorina Taban ◽

Cristian Suciu ◽

Codruta Lazureanu ◽

Alis Dema

Keyword(s):

Histopathological Examination ◽

Intraepithelial Neoplasia ◽

Heterotopic Pancreas ◽

Pancreatic Intraepithelial Neoplasia ◽

Unique Case ◽

Intraductal Papillary Mucinous Neoplasms ◽

Pancreatic Endocrine Neoplasm ◽

First Case ◽

Mucinous Neoplasms

We hereby present the case of a 58-year-old male who underwent a total gastrectomy for gastric neoplasm. During the surgery, a tumor mass in the jejunum was identified, considered as metastasis, and resected. The histopathological examination of the jejunal lesion showed ectopic pancreas. In this area, two pathological distinct lesions were identified, one histologically compatible with pancreatic intraepithelial neoplasia (PanIN) type 2 lesion and the other with morphologic criteria for endocrine microadenoma. To our knowledge, this is the first case that evidences the presence of a concomitant premalignant exocrine lesion and benign endocrine lesion in a heterotopic pancreas (HP).Abbreviations: HP: heterotopic pancreas; IPMN: Intraductal Papillary Mucinous Neoplasms; PanIN: Pancreatic Intraepithelial Neoplasia; PEN: pancreatic endocrine neoplasm.

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Primary stromal sarcoma of breast: A case report and literature review

Breast Disease ◽

10.3233/bd-201012 ◽

2021 ◽

pp. 1-7

Author(s):

Mohammad Al-Wiswasy ◽

Mahmoud Al-Balas ◽

Raith Al-Saffar ◽

Hamzeh Al-Balas

Keyword(s):

Malignant Tumors ◽

Histopathological Examination ◽

Prognostic Significance ◽

Total Duration ◽

Young Patients ◽

Soft Tissue Sarcomas ◽

Breast Mass ◽

Resection Margins ◽

Stromal Sarcoma ◽

First Case

INTRODUCTION: Primary breast sarcoma (PBS) was first described in 1887 as a rare heterogeneous neoplasm arising from the mesenchymal tissue of the mammary gland accounting for less than 5% of all body soft-tissue sarcomas and less than 0.1% of all malignant tumors of the breast. CASE PRESENTATION: A 31-year old lady presented with left breast mass which she felt four years before during which the mass increased in size from 1 to 6 cm in the largest diameter, diagnosed clinically as a benign fibroadenoma without any further cytological or histopathological confirmation. Histopathological examination of the excised breast mass reveals undifferentiated, primary stromal sarcoma of the breast (PSSB), which was followed by mastectomy three weeks later with reconstructive breast surgery with a total duration of follow-up of 3 years thereafter. This is the first case of PSSB reported in Jordan. DISCUSSION/CONCLUSION: PSSB is the generic term given to malignant breast tumors thought to arise from the specialized mesenchymal stroma of the breast but lacking an epithelial component with a phylloides pattern. PSSB is difficult to diagnose preoperatively due to its rarity and inadequate imaging methods to establish an exact diagnosis. The histology of the patient mass may be the leading factor for the management of these tumors. Even in very young patients, a progressively growing breast mass should alert the clinician to investigate for malignancy and verify the results by biopsy. Surgery with adequate resection margins represents the only potentially curative modality with prognostic significance. Adjuvant chemotherapy and radiotherapy are not very beneficial. The prognosis is dismal for patients with lymph node involvement and the size of the tumor has a lesser bearing on the outcome.

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Fetus in fetu: two case reports from North African country

Egyptian Pediatric Association Gazette ◽

10.1186/s43054-020-00049-5 ◽

2021 ◽

Vol 69 (1) ◽

Author(s):

Moutaz Ragab ◽

Omar Nagy Abdelhakeem ◽

Omar Mansour ◽

Mai Gad ◽

Hesham Anwar Hussein

Keyword(s):

Histopathological Examination ◽

Case Reports ◽

Abdominal Mass ◽

Mature Teratoma ◽

Surgical Techniques ◽

Rare Condition ◽

Mass Effect ◽

Fetus In Fetu ◽

Vascular Connection ◽

First Case

Abstract Background Fetus in fetu is a rare congenital anomaly. The exact etiology is unclear; one of the mostly accepted theories is the occurrence of an embryological insult occurring in a diamniotic monochorionic twin leading to asymmetrical division of the blastocyst mass. Commonly, they present in the infancy with clinical picture related to their mass effect. About 80% of cases are in the abdomen retroperitoneally. Case presentation We present two cases of this rare condition. The first case was for a 10-year-old girl that presented with anemia and abdominal mass, while the second case was for a 4-month-old boy that was diagnosed antenatally by ultrasound. Both cases had vertebrae, recognizable fetal organs, and skin coverage. Both had a distinct sac. The second case had a vascular connection with the host arising from the superior mesenteric artery. Both cases were intra-abdominal and showed normal levels of alpha-fetoprotein. Histopathological examination revealed elements from the three germ layers without any evidence of immature cells ruling out teratoma as a differential diagnosis. Conclusions Owing to its rarity, fetus in fetu requires a high degree of suspicion and meticulous surgical techniques to avoid either injury of the adjacent vital structures or bleeding from the main blood supply connection to the host. It should be differentiated from mature teratoma.

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In vivo evaluation of a recombinant N-acylhomoserine lactonase formulated in a hydrogel using a murine model infected with MDR Pseudomonas aeruginosa clinical isolate, CCASUP2

AMB Express ◽

10.1186/s13568-021-01269-7 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Masarra M. Sakr ◽

Walid F. Elkhatib ◽

Khaled M. Aboshanab ◽

Eman M. Mantawy ◽

Mahmoud A. Yassien ◽

...

Keyword(s):

Survival Rate ◽

Murine Model ◽

Histopathological Examination ◽

Healing Process ◽

Bacterial Count ◽

Treated Group ◽

Control Group ◽

In Vivo Evaluation ◽

Systemic Spread

AbstractFailure in the treatment of P. aeruginosa, due to its broad spectrum of resistance, has been associated with increased patient mortality. One alternative approach for infection control is quorum quenching which was found to decrease virulence of such pathogen. In this study, the efficiency of a recombinant Ahl-1 lactonase formulated as a hydrogel was investigated to control the infection of multidrug resistant (MDR) P. aeruginosa infected burn using a murine model. The recombinant N-acylhomoserine lactonase (Ahl-1) was formulated as a hydrogel. To test its ability to control the infection of MDR P. aeruginosa, a thermal injury model was used. Survival rate, and systemic spread of the infection were evaluated. Histopathological examination of the animal dorsal skin was also done for monitoring the healing and cellular changes at the site of infection. Survival rate in the treated group was 100% relative to 40% in the control group. A decrease of up to 3 logs of bacterial count in the blood samples of the treated animals relative to the control group and a decrease of up to 4 logs and 2.3 logs of bacteria in lung and liver samples, respectively were observed. Histopathological examination revealed more enhanced healing process in the treated group. Accordingly, by promoting healing of infected MDR P. aeruginosa burn and by reducing systemic spread of the infection as well as decreasing mortality rate, Ahl-1 hydrogel application is a promising strategy that can be used to combat and control P. aeruginosa burn infections.

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Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle: A Case of Relapse Treated with Proton Beam Therapy

Diagnostics ◽

10.3390/diagnostics11050903 ◽

2021 ◽

Vol 11 (5) ◽

pp. 903

Author(s):

Antonella Cacchione ◽

Angela Mastronuzzi ◽

Andrea Carai ◽

Giovanna Stefania Colafati ◽

Francesca Diomedi-Camassei ◽

...

Keyword(s):

Adjuvant Therapy ◽

Proton Beam ◽

Fourth Ventricle ◽

Histopathological Examination ◽

Local Level ◽

Proton Beam Therapy ◽

Disease Recurrence ◽

Glioneuronal Tumor ◽

First Case ◽

Cystic Component

Rosette-forming glioneuronal tumors (RGNTs) are rare, grade I, central nervous system (CNS) tumors typically localized to the fourth ventricle. We describe a 9-year-old girl with dizziness and occipital headache. A magnetic resonance imaging (MRI) revealed a large hypodense posterior fossa mass lesion in relation to the vermis, with cystic component. Surgical resection of the tumor was performed. A RGNT diagnosis was made at the histopathological examination. During follow-up, the patient experienced a first relapse, which was again surgically removed. Eight months after, MRI documented a second recurrence at the local level. She was a candidate for the proton beam therapy (PBT) program. Three years after the end of PBT, the patient had no evidence of disease recurrence. This report underlines that, although RGNTs are commonly associated with an indolent course, they may have the potential for aggressive behavior, suggesting the need for treatment in addition to surgery. Controversy exists in the literature regarding effective management of RGNTs. Chemotherapy and radiation are used as adjuvant therapy, but their efficacy management has not been adequately described in the literature. This is the first case report published in which PBT was proposed for adjuvant therapy in place of chemotherapy in RGNT relapse.

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Calcaneal Reconstruction With Free Deep Circumflex Iliac Artery Osseocutaneous Flap Following Aggressive Benign Bone Tumor Resection

Foot & Ankle International ◽

10.1177/10711007211025280 ◽

2021 ◽

pp. 107110072110252

Author(s):

Mohamad Aizat Rosli ◽

Wan Faisham Wan Ismail ◽

Wan Azman Wan Sulaiman ◽

Nor Azman Mat Zin ◽

Syurahbil Abdul Halim ◽

...

Keyword(s):

Iliac Artery ◽

Donor Site ◽

Tumor Resection ◽

Case Series ◽

Benign Tumors ◽

Reconstruction Method ◽

Short Term ◽

Deep Circumflex Iliac Artery ◽

First Case ◽

Donor Site Pain

Background: Advances in limb-salvage techniques have made total calcanectomy and primary reconstruction possible in managing calcaneal aggressive benign tumors and selected cases of intraosseous malignant tumors. However, there is still no consensus on the operative approach, oncologic margin, and the best reconstruction method to date. These 2 cases describe our experience in calcaneal reconstruction with the free deep circumflex iliac artery (DCIA) osseocutaneous flap in benign aggressive calcaneal tumors. Methods: We reported 2 consecutive male and female patients, with an average age of 25 years (age 19 and 31, respectively), who underwent total calcanectomy and primary calcaneal reconstruction with the free DCIA osseocutaneous flaps for calcaneal chondroblastoma and giant cell tumor. A marginal resection of the entire calcaneus through the subtalar and calcaneocuboid joints (intra-articular approach) was performed in the first case and a wide local resection leaving 1 cm normal calcaneal bone margin anterosuperiorly (intraosseous approach) was performed in the second case. Results: The follow-up period averaged 48 months. Negative oncologic margins were achieved in both cases. The first case was complicated with venous thrombosis; however, the graft remained viable after emergency reexploration. Normal foot function was restored with good solid osseous union and bony hypertrophy observed. Both patients achieved good short-term functional and aesthetic outcomes with no donor site pain or disability. No local recurrence was reported either. Conclusion: Primary calcaneal reconstruction with the free DCIA osseocutaneous flap can lead to good short-term functional and aesthetic outcomes. Level of Evidence: Level IV, case series.

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BRAF-MAD1L1 and BRAF-ZC3H7A: novel gene fusion in Rhabdomyosarcoma and Lung adenocarcinoma

10.21203/rs.3.rs-368083/v1 ◽

2021 ◽

Author(s):

Jiang Da ◽

Hui Jin ◽

Xinliang Zhou ◽

Shaoshuang Fan ◽

Mian Xu ◽

...

Keyword(s):

Lung Adenocarcinoma ◽

Gene Fusion ◽

Tumor Resection ◽

Kinase Domain ◽

Rapid Progression ◽

First Line ◽

Case Presentation ◽

First Case ◽

Exon 11 ◽

Domain Exon

Abstract Background: Rhabdomyosarcoma (RMS) and lung adenocarcinoma (LADC) epitomizes the success of cancer prevention by the development of conventional therapy, but huge challenges remain in the therapy of advanced diseases.Case presentation: We reported two cases of novel BRAF gene fusion. The first case was a 34-year-old female with RMS harboring a BRAF-MAD1L1 fusion. She suffered tumor resection, recurrence and rapid progression. The second case was a 72-year-old female with LADC harboring a BRAF-ZC3H7A fusion, and she gained rapid progression after receiving a first-line course of chemotherapy.Conclusions: These two BRAF fusions retain the intact BRAF kinase domain (exon 11-18) and showed poor prognosis in RMS and LADC.

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