Microcystic Adnexal Carcinoma. A Rare Entity.

Background: Microcystic adnexal carcinoma is a rare malignant tumour derived from sweat glands, locally aggressive, but with low rate of lymphatic or metastatic spread. Tends to affect the deep dermis, without affection of epidermis. Surgery remains as the first line treatment. Case Report: We present a case of a 46-year-old woman with a slow growing lesion of the upper lip, with biopsy diagnosis of microcystic adnexal carcinoma. She underwent a resection and reconstruction with local advancement flaps. The final anatomopathological study showed an adnexal epithelial neoplasm with imprecise borders, poorly delimited, non-encapsulated, growing in plaque-like formation from the superficial dermis into the adipose tissue, perineural invasion, without epidermal infiltration. Discussion: It is an extremely rare malignant tumour, appearing as a solitary papule or plaque affecting the central face, that often affect middle-aged caucasic, female patients. Usual local aggressive nature, characterized by small nests and strands of cells in deep dermis and perineural-invasion images, absent in superficial tissue. An incisional biopsy is need to make a correct diagnosis. Due to its rarity there is no consensus on the best management and follow-up. The microcystic adnexal carcinoma should be taken into consideration in the differential diagnosis. Key words:Microcystic adnexal carcinoma, lip, histopathology.

Recurrent Pediatric Extranasopharyngeal Angiofibroma of the Epiglottis: Case Report.

Angiofibroma is a non-encapsulated, highly vascular tumor that usually originates in the nasopharynx. Laryngeal cases of extranasopharyngeal angiofibroma (ENA) are a very rare pathology, especially in children. Only eight ENA laryngeal cases have been described in the literature, and only one of them is a pediatric case. In this report we present an 11-year-old child with epiglottic ENA resected with transoral endoscopic ultrasonic surgery (TOUSS) with review of the literature. Because of reccurrence after five months he underwent re-excision with CO2 laser. Recurrences in ENA are infrequent, but as demonstrated in our case, close endoscopic follow-up is mandatory in this location. Endoscopic hemostatic procedures like TOUSS and CO2 laser ensure bloodless surgery for the management of this type of vascular laryngeal tumors.

A Clear Cell Sarcoma Case: A Diagnostic and Treatment Challenge, with a Promising Response to Trabectedin.

Introduction: Clear cell sarcoma (CCS) is a rare and aggressive soft tissue sarcoma. CCS is characterized by the translocation t(12;22) (q13;q12), involving the fusion of EWSR1 and ATF1 genes, and less frequently the fusion gene EWSR1-CREB1. Usually, CCSs are considered poorly responsive to conventional chemotherapy. However, trabectedin has shown activity against translocation-related sarcomas. Furthermore, preclinical results suggest that trabectedin is a promising antitumor agent for CCS, potentially inducing melanocytic differentiation. Case Presentation: We report the case of a challenging anatomopathological diagnosis in a patient with an aggressive metastatic CCS. Following the diagnosis of CCS, the patient experienced a clinical and radiological tumor response to trabectedin after four lines of treatment. Conclusion: This is a novel report of CCS treated with trabectedin that resulted in a partial response and suggests the need for further research on trabectedin as a therapeutic option for CCS.

Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment.

BACKGROUND: Solitary fibrous tumor is an unusual fibroblastic mesenchymal neoplasm typically described in the pleura. It may appear anywhere with a varied anatomic distribution and essentially it can develop from any soft tissue or visceral location. Its course is usually indolent and it rarely causes distant metastases, so it has a prolonged survival rate. It sometimes presents itself as a disseminate disease being the liver the most frequently involved location. In these occasions, the management should be discussed in a multidisciplinary tumor committee formed by surgeons, oncologists and radiologists. Surgery remains the gold standard for treatment. CASE REPRESENTATION: We present the case of a woman with a tumor in the left abdominal wall and bilobar massive liver metastases, both locations histologically diagnosed as solitary fibrous tumor. She receives biological treatment for a severe case of Crohn´s disease. Evaluated in a multidisciplinary committee, surgery was recommended for both the primary lesion and the liver metastases. The hepatobiliary surgeons considered a two-stage hepatectomy with portal vein embolization (PVE) as the best strategy. After the first procedure consisting in cleaning the left hepatic lobe followed by PVE the future liver remnant volume (FLRV) was considered inadequate, so the patient was also treated with right transarterial radioembolizacion with yttrium 90 (TARE-Y90) intending a double goal: to treat the tumor and to increased the FLRV. Furthermore, a severe flare of Crohn´s disease forced us to intensify the patient's treatment with the addition of biological agents (infliximab and adalimumab) until complete remission of the symptoms. The second stage of the liver surgery had to be postponed for more than 6 months and could finally be carried out without complications, achieving an R0 resection. The postoperative course was uneventful and the follow up has showed no recurrence to date. CONCLUSION: Solitary fibrous tumours with extensive liver metastases are infrequent but when they appear modern surgical strategies like two stage hepatectomy are the treatment of choice and must be carried out by specialised units. The therapeutic decisions should be guided by a multidisciplinary committee.

Cytologic features of the normal pineal gland of adults.

It is well known that the histology of normal pineal gland may resemble not only pineal tumors but also gliomas, owing to its cellularity which is much greater than that of normal white or gray matter. Our recent experience with a case in which part of a normal gland was submitted for intraoperative consultation, together with the scarcity of cytologic descriptions, led us to perform a cyto-histologic correlation study. In addition to the intraoperative case, we collected five pineal glands from consecutive adult autopsies. During the squash procedure, we often noted the presence of calcified grains. Smears were hypercellular, distributed in tissue fibrillary fragments and as numerous single cells, with crystalline structures. Pineal gland cells (pineocytes) were large, round, epithelioid with ill-defined cytoplasms and moderate nuclear pleomorphism. Spindle cells with greater fibrillary quality were less common. One of the most remarkable findings seen in all cases was the presence of cytoplasmic pigment. Histological evaluation and immunohistochemical staining confirmed that the tissue was normal pineal gland. The histology showed a characteristic lobular aspect and frequent corpora arenacea. The pigment seen cytologically was also encountered in histology and corresponded to lipofuscin. Cytologic features of the pineal gland are peculiar when compared to other normal structures of the central nervous system. These features correlate closely with what is seen on histology. In an adequate clinical context, and in combination with frozen sections, cytology allows a specific recognition of the pineal gland during intraoperative pathologic consultations.