scholarly journals Wide Excision of a Retroperitoneal Liposarcoma with En Bloc Ureterectomy and Renal Salvage by Autotransplantation

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Siegfredo R. Paloyo ◽  
Arjel D. Ramirez ◽  
Ferri P. David-Paloyo ◽  
Rodney B. Dofitas

Liposarcoma is a malignant mesenchymal neoplasm composed of adipose tissue with varying degrees of atypia. These tumors grow slowly and may reach an enormous size particularly if located in the retroperitoneum. We report a 40-year-old male with a 6-month history of gradual abdominal enlargement. Computed tomography (CT) of the abdomen showed a huge retroperitoneal mass with characteristic features consistent with liposarcoma. On laparotomy, the mass was noted to be encasing the right ureter for which a wide excision with en bloc ureterectomy and subsequent renal autotransplantation for organ preservation was done. Post-operative course was uneventful with excellent outcome after 6 months of follow-up. Final histopathologic diagnosis was low-grade, well differentiated liposarcoma, which has favorable prognosis following radical surgery. This was the first report of such a case in the Philippines.

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Koto Fujiishi ◽  
Shigenori Nagata ◽  
Rieko Kano ◽  
Chiaki Kubo ◽  
Maasa Shirayanagi ◽  
...  

Abstract Background Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, recently specified as harboring JAZF1–SUZ12 rearrangement. Typical JAZF1–SUZ12 ESS is slow growing, in which high uptake of fluorodeoxyglucose (FDG) on positron emission tomography (PET) and subserosal masses are quite unusual. Case presentation A 69-year-old Japanese woman complained of urinary incontinence. Pelvic magnetic resonance imaging showed uterine lesions composed of (1) a 9 × 8 × 7-cm mass protruding from the right-anterior wall, (2) a 4.5-cm mass attached to the right-posterior wall, and (3) a 6.5-cm intramural mass in the fundus. FDG-PET demonstrated maximum standardized uptake value of 13.28 confined to the two subserosal masses (1 & 2) in contrast to no uptake of the intramural mass (3). She was diagnosed with a high-grade uterine sarcoma concomitant with leiomyomas and underwent total hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. The removed uterus had three tumors—two in the right-anterior and right-posterior subserosa, respectively, and the remaining in the fundal myometrium. Microscopically, the three tumors shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which neither round-cell component, pleomorphism, nor high mitotic activity was recognized. Nuclear cyclin D1 immunostaining was identified 50% of neoplastic cells in the two subserosal tumors (1 &2) whereas < 1% positive cells in the intramural component (3). Reverse transcriptase-polymerase chain reaction showed the same-sized electrophoretic bands indicating JAZF1–SUZ12 gene fusion shared by the three uterine tumors and a focal tumor extension into the extrauterine vein. The patient is alive without evidence of recurrence at 14 months after surgery. Conclusions Pathologists and clinicians should not exclude the possibility of JAZF1–SUZ12 ESS even when uterine subserosal masses demonstrate extraordinary FDG uptake on PET. Molecular analysis is helpful for diagnostic confirmation of JAZF1–SUZ12 ESS with a complex growth pattern.


2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Nicolas Macagno ◽  
Stéphane Fuentes ◽  
Gonzague de Pinieux ◽  
André Maues de Paula ◽  
Sébastien Salas ◽  
...  

Despite being one of the most frequent soft-tissue sarcomas, well-differentiated liposarcoma has never been reported near the spine. The authors present the case of a 67-year-old man with progressive history of back pain. Physical examination revealed a mass located within the right paravertebral muscles. MR and CT imaging showed a heavily ossified central mass surrounded by a peripheral fatty component. No connection with the underlying bone was detected on imagery and during surgery. After surgical resection, histopathological examination revealed a tumor harboring combined features of well-differentiated liposarcoma and low-grade osteosarcoma. Tumor cells displayed overexpression of MDM2, CDK4, and P16 by immunohistochemistry and CGH revealed amplification of 12q13-15 as the only genetic imbalance. MDM2 FISH analysis was performed but was inconclusive. The pathological, immunohistochemical, and genetic features, the differential diagnoses, and the therapeutic management of this unusual tumor are discussed. No complementary treatment was performed initially. Following first treatment, two recurrences occurred 6 and 9 years later, both displaying histological features similar to the first occurrence. Radiotherapy was started after the second recurrence. Follow-up shows no evidence of disease 11 years after initial diagnosis. This case was unusual due to the paravertebral location of the tumor and its divergent differentiation.


2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Jason E. Cohn ◽  
Hilary M. Caruso Sales ◽  
Giang Huong Nguyen ◽  
Harvey Spector ◽  
Kenneth Briskin

Keratoacanthoma (KA) is a benign epithelial tumor that typically presents as a firm, cone-shaped, flesh-colored nodule with a central horn-filled crater. KA is considered to be a low-grade variant of squamous cell carcinoma (SCC). We report a rare case of a 72-year-old male who presented with a KA involving the nasal septum, possibly related to ranibizumab use. A flesh-colored lesion on the right anterior nasal septum lesion was visualized on examination. Histologic examination revealed a well-circumscribed, dome-shaped central crater filled with keratin, well-differentiated squamous epithelium with ground-glass cytoplasm with pushing margins, and intraepithelial microabscesses establishing the diagnosis of KA. KA of the nasal septum has only been reported once in the literature. This case is unusual because it normally presents on sun-exposed areas. Additionally, this patient was taking ranibizumab, a vascular endothelial growth factor (VEGF) inhibitor for macular degeneration. Despite ranibizumab not being directly linked to precancerous and cancerous skin lesions, agents in this medication class have been. Although it is difficult to prove associations in this isolated case, the role of ranibizumab causing cutaneous lesions should be further investigated.


2017 ◽  
Vol 12 (4) ◽  
pp. 572-575 ◽  
Author(s):  
Takashi Anzai ◽  
Tsuyoshi Saito ◽  
Sho Tsuyama ◽  
Miri Toh ◽  
Katsuhisa Ikeda ◽  
...  

Abstract Glomangiopericytoma (GPC) is a rare sinonasal perivascular tumor that accounts for < 0.5–1% of all sinonasal tumors. GPC is categorized as a low-grade neoplasm with borderline malignancy and a tendency of local recurrence. GPC is a rare mesenchymal neoplasm characterized by the perivascular proliferation of tumor cells, and it requires being distinguished from solitary fibrous tumors. Here, we report a case of GPC in a 68-year-old male patient who presented at the emergency room of our hospital with a complaint of sudden epistaxis. A small, reddish, protruding tumor was observed on the right nasal septum. A biopsy revealed a possible perivascular tumor such as a GPC or solitary fibrous tumor. Thus, we performed complete resection with endoscopic surgery. The size of the resected tumor was 12 × 5 mm, and it showed a uniform proliferation of oval-to-short spindle-shaped cells with slightly branching vascular structures. The tumor cells showed minimal cytologic atypia and there were an average of 3 mitoses in 10 high power fields. Necrosis was not observed. The tumor cells showed strong and diffuse nuclear immunostaining with beta catenin and were negative with STAT6, CD34 and bcl-2. The MIB-1 labeling index was approximately 5%. Genetic testing revealed CTNNB1 mutation (p.S33C). Thus, a diagnosis of low grade GPC was made on the biopsy and the patient could be successfully treated with endoscopic resection.


2019 ◽  
Vol 12 (3) ◽  
pp. e228622
Author(s):  
Muhammad Ibaad ur Rehman Alvi ◽  
Omar Pathmanaban ◽  
Raj Bhalla ◽  
Navin Mani

A well 74 -year-old woman presented to routine ophthalmology clinic instituted following treatment of a frontoethmoidal sarcoma initially excised in 1989 and diagnosed then as a sinus mucosal melanoma. At review in ophthalmology clinic, a reduction in right visual fields was noted. CT scan showed recurrence of a mass now involving the frontoethmoidal region, frontal sinus and abutting the cribiform plate. Endoscopic biopsy confirmed the recurrence as a low-grade biphenotypic sarcoma. This was discussed at the sarcoma multidisciplinary meeting. Using a three-dimensional printed model of the patients skull for planning, primary surgery with craniofacial resection combining intracranial and transfacial approaches with reconstruction was decided on. The implications of no treatment would be tumour involvement of the dura and brain as well as the right only-seeing eye. Craniofacial surgery would involve otolaryngolical, neurosurgical and maxillofacial multispecialty involvement and close teamwork. The goal was en bloc excision with negative surgical margins.


2005 ◽  
Vol 71 (3) ◽  
pp. 191-193
Author(s):  
S.A. Mclaughlin ◽  
T.M. Schmitt ◽  
K.L. Huguet ◽  
D.M. Menke ◽  
J.H. Nguyen

Adrenal masses have varying presentations. Most commonly, adrenal masses are discovered incidentally on CT or MRI during an evaluation for an unrelated complaint. Although the majority of these are nonfunctional cortical adenomas, hormonally active tumors and adrenocortical carcinoma must also be considered in the differential diagnosis. Rarely, retroperitoneal tumors may mimic an adrenal mass. We report a case of a 49-year-old man with anemia and weight loss who was found to have a large retroperitoneal mass arising from the adrenal gland. Surgical treatment involved en bloc resection of the right kidney, adrenal gland, segments 7 and 8 of the liver, and a portion of the right hemidiaphragm. Final pathology revealed a low-grade myofibrosarcoma. We believe that this is the first case report of a myofibrosarcoma of the adrenal gland. Myofibrosarcomas are rare malignant tumors composed of myofibroblasts that arise from the deep soft tissues. These tumors have a predilection for the head and neck, trunk, or extremities. Myofibrosarcomas can be differentiated from other sarcomas by immunohistochemical staining and pathologic features. We will briefly discuss the workup of an adrenal mass and focus on the diagnosis of myofibrosarcoma.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
My-Linh T. Nguyen ◽  
Christopher J. LaFargue ◽  
Tarah L. Pua ◽  
Sean S. Tedjarati

Most grade 1 endometrioid endometrial cancers are confined to the uterus at the time of diagnosis and confer a good prognosis. Rarely will a grade 1 endometrioid endometrial carcinoma present with distant metastasis, especially to the bone. We present the case of a 56-year-old woman with postmenopausal bleeding and right hip pain due to metastatic grade 1 endometrioid uterine cancer invading into the right ischium. We discuss treatment options as well as provide a review of prior published reports on bony metastasis in grade 1 endometrioid endometrial cancers. To date, this case is one of 10 others which demonstrates that even a well-differentiated, low-grade endometrioid endometrial carcinoma can progress in a highly aggressive manner.


2015 ◽  
Vol 53 (200) ◽  
pp. 301-303 ◽  
Author(s):  
Ramesh Singh Bhandari ◽  
Paleswan Joshi Lakhey ◽  
Parsu Ram Mishra

A 57 year old male presented to our outpatient clinic with history of on and off melena, weight loss and decreasing appetite for 10 months duration and a noticeable mass on the right upper quadrant. Abdominal examination revealed an intra-abdominal lump in right upper quadrant which was subsequently evaluated by colonoscopy, which revealed an ulcero-proliferative growth in the hepatic flexure and the biopsy from it confirmed well-differentiated adenocarcinoma. Contrast enhanced computed tomography demonstrated hepatic flexure mass with possible invasion into adjacent duodenum without features of advanced disease. After completion of necessary preoperative assessment and investigations, patient was explored with curative intent and underwent extended right hemicolectomy with en bloc pancreaticodudenectomy. Patient was discharged on 10th postoperative day and at 14 months follows up; he was doing well without any evidence of recurrence. Keywords: colectomy; locally advanced; pancreaticoduodenectomy.


2019 ◽  
Vol 27 (8) ◽  
pp. 868-871 ◽  
Author(s):  
David Creytens ◽  
Thomas Mentzel ◽  
Liesbeth Ferdinande ◽  
Joost van Gorp ◽  
Jo Van Dorpe ◽  
...  

We report the case of a “fat-rich” (spindle cell-poor) variant of an atypical spindle cell/pleomorphic lipomatous tumor in a 63-year-old female patient presenting with a firm, painless soft tissue mass on the right hip. Atypical spindle cell/pleomorphic lipomatous tumor is a very recently described low-grade adipocytic neoplasm, which occurs predominantly in adults with a predilection for the limbs and limb girdles. In the present case, the diagnosis of an atypical spindle cell/pleomorphic lipomatous tumor was challenging because the tumor was almost exclusively composed of an atypical adipocytic component (resembling “classical” atypical lipomatous tumor/well-differentiated tumor) with only very focal presence of the diagnostic atypical morphologic features (atypical spindle-shaped cells, floret-like multinucleated cells, and “bizarre” pleomorphic [multinucleated] cells). The possibility of a “classical” atypical lipomatous tumor/well-differentiated liposarcoma was ruled out by immunohistochemistry (lack of MDM2 expression and loss of Rb expression) and molecular genetic testing (no amplification of MDM2 and presence of monoallelic deletion of RB1). Another interesting morphologic observation in this case was the striking perivascular location of the atypical spindle/pleomorphic cells in some areas (so-called “pericytic mimicry”). To our knowledge, pericytic mimicry has not been reported in the setting of an atypical spindle cell/pleomorphic lipomatous tumor.


Author(s):  
T. Ramani Devi ◽  
D. Sangavi ◽  
A. Deepika

Angiomyxoma is a rare slow-growing, benign low-grade tumor occurring in women of reproductive age group which is known for its recurrence. The symptoms are variable. Mrs. K. aged 33 years, reached our outpatient department (OPD) with complaints of painless swelling on the right labial region which she was feeling uncomfortable while sitting. Mass was found to be mobile with no evidence of inflammatory change. The location of the mass made us think about, Bartholin’s cyst and hence surgical excision of the mass was done. The whole mass was found to be lying below the fat in the right labial region and wide excision was completed. The histopathology of the mass was reported as angiomyxoma. Angiomyxoma arises from the mesenchymal tissue and it is locally invasive with high recurrence rate. It is more common in females. Mostly seen in the vulvovaginal, pelvic cavity and perineum. The lesion can reach huge size. It has to be differentiated from gynecologic malignancies, cyst, abscess and hernia. Histology along with immune-histochemistry can confirm the lesion. Wide excision is the mode of treatment. Incomplete excision can lead to relapse. Relapse can happen in 35-72% of the cases. Local recurrence may occur between 2 months to 15 years following initial diagnosis. Hence, follow up is essential. Angiomyxoma is a very rare condition and only around 250 cases have been reported in the world literature. It has to be differentiated from other benign conditions. Diagnosed by non-invasive techniques like ultrasound sonography (USG), magnetic resonance imaging (MRI) and computed tomography (CT). Wide excision is the treatment of choice. Long term follow up is needed as recurrences are high.


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