Benign renal schwannoma: Case report and literature review.

Renal schwannoma is a very infrequent tumor. It is usually benign and it does not have any specific symptoms or imaging characteristics. Its final diagnosis is usually made after surgery. We present a 66 year-old-man that was referred to our center after the casual finding of a renal mass. With a suspected diagnosis of a renal cell carcinoma, a partial nephrectomy was performed. The histological study revealed the final diagnosis of a benign renal schwannoma.

[The value of immunohistochemistry using SMARCA4/BRG1 in the diagnosis of small cell ovarian carcinoma hypercalcemic type. A report of two cases]. / Valor de la determinación inmunohistoquímica de SMARCA4/BRG1 en el diagnóstico del carcinoma de ovario de célula pequeña variante hipercalcémica. Presentación de dos casos.

Small cell carcinoma of ovary-hypercalcemic type is an undifferentiated carcinoma. We describe two cases in women aged 32 and 29. Both presented with large masses and complete surgical extirpation was impossible. Histologically, the images were similar, with diffuse cell proliferation, accompanied by the presence of follicle-like spaces. In both cases it was necessary to make a differential diagnosis with entities such as adult or juvenile granulosa cell tumour, small cell carcinoma of pulmonary type, dysgerminoma and even peripheral neuroectodermal tumour. The absence of SMARCA4/BRG1 immunostaining proved very useful in the diagnosis of hypercalcemic small cell ovarian carcinoma.

Arteriovenous Malformations of the Temporalis Muscle: A Comprehensive Review.

Arteriovenous malformations of the temporalis muscle (TM-AVMs) are rare lesions commonly considered as a variant of scalp AVMs. A study was designed in order to analyze TM-AVMs features and to confirm them as a certain entity different from scalp AVMs. Seven TM-AVMs cases (5 from literature plus 2 from our database) were studied. Clinical, epidemiological, radiological, and therapeutic data were analyzed and compared to common scalp AVMs features. All TM-AVMs presented as large soft tumor-like masses on the temporal region (mean diameter: 6 cm). Pain was referred in half of cases, while overlying cutaneous stigmas were described only once. No masticatory malfunction was reported. TM-AVMs tend to appear in young patients (mean: 29 yr), commonly as longstanding and slow-evolving lesions (mean evolution time before diagnosis: 5 yr). All cases displayed a diffuse slow-flow blush on angiogram. All TM-AVMs were completely located within the TM, with no involvement of scalp or bone. The pathological analysis of all cases demonstrated the presence of striate muscular bundles intermingled with fairly mature arterial and venous vessels. No clearly distorted vessel was found within the TM-AVMs. Surgery was performed for all cases with complete removal. Preoperative embolization was done in 4 cases to reduce blood loss during surgery. No clinical/radiological recurrence was reported. Opposite to TM-AVMs, scalp AVMs commonly present as high-flow lesions with no evident tumor mass, mainly located in the subcutaneous tissue. TM-AVMs present clearly distinct features compared to scalp AVMs, for which they should be considered as a certain pathological entity and not as a variant of scalp AVMs.

An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review.

We report the case of a 67 year old male who presented with a nine year history of a gastric ulcer with symptoms of hematemesis and melena. Histological analysis identified fibrotic lesions and the accumulation of immunoglobulin G4-positive plasma cells with no evidence of malignancy. The lesion extended into the pancreas, where histological lesions and gastric lesions were also observed. This is a case of an ulcerated gastric ulcer and pseudo-tumor with pancreatic affection that is associated with immunoglobulin G4-related disease.

Endometrial stromal nodule of the vaginal wall with a review of vulvovaginal endometrial stromal neoplasms.

•It is reported the first endometrial stromal nodule (ESN) in the vagina.•This is an excepcionall ESN because it was not associated with endometriosis•It was successfully treated by local resection.•Primary vulvovaginal endometrial stromal neoplasms are rare (only 5 reported).

Chronic spontaneous diploic hematoma.

The authors report the case of a 23-year-old woman with café-au-lait spots and axillary and inguinal freckling who presented with a diploic chronic spontaneous hematoma of the left parietal bone. To the authors' knowledge, this case represents the first description of a diploic hematoma in a patient with stigmata of neurofibromatosis Type 1 unrelated to head trauma. Plain skull radiography showed an osteolytic lesion with well-circumscribed margins, corresponding to the hematoma, together with exuberant perilesional vascular markings. Angiography demonstrated an incidental aneurysm of the left supraclinoidal internal carotid artery and an unusual cortical venous drainage toward the diploic vessels. The blood flow of these vessels on the right hemicranium was sluggish and exhibited enlarged diploic venous lacunas. The authors hypothesize that the hematoma was formed by both an abnormal venous drainage toward the diploic vascular net, together with a vasculopathy that caused stenosis and obstruction of the normal drainage pathways from these vessels.

Spinal dermal sinus and pseudo-dermal sinus tracts: two different entities.

BACKGROUND: Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD. We termed this lesion pseudo-dermal sinus tract. PATIENTS AND METHODS: We reviewed clinical features of 20 patients with spinal skin orifices. The patients were classified into two categories: group 1 comprised children with true DST and group 2 included patients with skin dimples resembling a DST. AIMS: The aim of this study was to analyze differential features of patients in these two groups as they behaved dissimilarly in regard to clinical significance and outcomes, especially those concerning infectious risk. RESULTS: Children of group 1 (n = 8) presented with tiny skin orifices and with superficial or deep infection. In contrast, group 2 (n = 12) mostly manifested with neurological symptoms. No patient in group 2 developed an infection preoperatively. In both groups, magnetic resonance showed tracts that ended in different structures. Histopathology of the lesions were dissimilar, those of group 1 being hollow tubular structures lined by epithelium while those of group 2 being solid tracts of fibrous connective tissue. CONCLUSIONS: DST constitutes a clinicopathological diagnosis. Although sharing some common cutaneous and neuroimaging findings, both groups behaved differently. Patients of group 1 tended to show up with infection requiring urgent surgery. Patients of group 2 often presented with neurological manifestations and skin lesions, but needed no immediate operation.

Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.

BACKGROUND: Embryonal central nervous system (CNS) tumors are currently classified into three types: medulloblastoma, atypical rhabdoid/teratoid tumors, and primitive neuroectodermal tumor (PNET). A distinctive subtype of PNET called "embryonal tumor with abundant neuropil and true rosettes" (ETANTR) was reported in 2000. DISCUSSION: ETANTR is a recently described variety of PNET that combines microscopic features of neuroblastoma and ependymoblastoma, demonstrating areas of fine fibrillary neuropil intermingled with cellular zones and ependymoblastic rosettes. It has been suggested that this neoplasm should be considered as a separate entity. ETANTR is an eminently pediatric tumor that has been reported exclusively in children younger than 4 years. ILLUSTRATIVE CASES: A 9-month-old girl underwent subtotal resection of a brainstem neoplasm. A 23-month-old girl was submitted to surgery for a frontoparietal tumor. In both instances, the histopathological diagnosis confirmed ETANTR. Both children were treated with chemotherapy and one with radiotherapy. Death in both patients occurred after a mean period of 10 months. CONCLUSIONS: By reporting these two new instances of ETANTR, we want to contribute to the knowledge of this highly malignant CNS embryonal neoplasm that occurs only in young children, given its present lethal prognosis, the scarcity of reported cases, and the lack of treatment guidelines.

Foreign body reactions causing spinal cord tethering: a case-based update.

BACKGROUND: Patients operated on for myelomeningocele (MMC) and lipomeningocele (LMC) can suffer from late functional worsening that, in many cases, is due to spinal cord tethering by the post-repair scarring process. ILLUSTRATIVE CASES: In this case-based update, we report two patients operated on for MMC and LMC, respectively, who presented the clinical manifestations of spinal cord tethering, which we attributed to severe foreign body reactions to the materials used at their primary back surgery. In the first case, the cause of the tethering was an intense fibrotic scar around the silk suture used at the initial MMC repair, while in the second one, it was due to a fibrotic mesh containing the lyophilized dural graft implanted for LMC surgery. After a search of the current literature, we were unable to find cases of spinal cord tethering related to excessive scarring after dural repair with foreign materials used for the surgical correction of spinal dysraphism. DISCUSSION AND CONCLUSIONS: The pathogenesis of spinal cord tethering in our instances closely relate with the histopathologically observed intense foreign body reaction around the materials used at the primary back surgery. We recommend avoiding as much as possible the use of foreign materials during the initial repair of spinal dysraphism.

Poorly differentiated primary monophasic synovial sarcoma of the lung.

Primary monophasic synovial sarcoma of the lung is rarely seen in clinical practice. We report the case of a 60-year-old male who underwent a left lower lobectomy for lung sarcoma. The patient received adjuvant therapy after surgery. Diagnosis was confirmed with SYT-SSX2 translocation detection.

Rhabdoid meningioma: a new subtype of malignant meningioma also apt to occur in children.

CASE REPORT: The case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM). DISCUSSION: Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients.

[Fatal evolution of a renal angiosarcoma]. / Evolución fatal de angiosarcoma renal.

OBJECTIVES: Report a new case of renal angiosarcoma treated by surgery and adjuvant chemotherapy with bad results. METHODS: 72-year-old male undergoing right nephrectomy for renal tumor. Pathology reports renal angiosarcoma. RESULTS: Three months after surgery patient refers lumbar pain and hemoptysis and CT scan reveals the existence of multiple bone and lung metastasis; a regimen of systemic chemotherapy with Doxorrubicine+ Ifosfamide was started without response; he died two months later. CONCLUSIONS: Primary renal angiosarcoma is very rare, with less than 10 cases in the literature before 1998, and it is always associated with bad prognosis. Diagnosis is based on immunohistochemical studies (antibodies against CD31, CD34 and factor VIII related antigen) to define the endothelial differentiation of the tumor. There is no experience to define the best therapeutic strategy against this entity.

Congenital giant melanocytic nevus with pigmented epithelioid cells: a variant of epithelioid blue nevus.

Epithelioid-cell blue nevus is an unusual cytologic variant of blue nevus that has been recently described mostly in patients with Carney complex, although the lesion may also appear in patients with no evidence of Carney complex. This variant of blue nevus is composed of melanin laden large polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive descent and, in contrast with the usual stromal changes in blue nevi, epithelioid-cell blue nevus exhibits no dermal fibrosis. This report describes a congenital giant melanocytic nevus with pigmented epithelioid cells located on the back of a 2-year-old male. The lesion was present at birth and the patient had no evidence of Carney complex. Histopathologically, the lesion consisted of a large and entirely intradermal melanocytic nevus composed of heavily pigmented epithelioid melanocytes involving the full-thickness of dermis, but extending also to the subcutaneous fat and underlying soft tissues. Immunohistochemically, epithelioid neoplastic melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A, NK1C3, and microphthalmia transcription factor (MiTF) antibodies. MIB-1 cellular proliferation marker was expressed in the nuclei of only a few scattered epithelioid melanocytes. This report demonstrates that epithelioid-cell blue nevus is a distinctive histopathologic variant of blue nevus that may also appear as a giant congenital melanocytic nevus.