Malignant solitary fibrous tumor of the lumbar spinal root mimicking schwannoma: a case report

Background contextMalignant solitary fibrous tumors (SFTs) arising from the spinal cord are extremely rare and poorly understood mesenchymal neoplasms. To date, only one malignant SFT located in the spinal canal of the sacrum has been described, but none arising from the lumbar nerve root have been reported. Although most SFTs with benign histological features can be treated by complete surgical excision alone, malignant SFTs may require adjuvant therapy. However, systemic chemotherapy and radiotherapy have not been shown effective in patients with malignant SFTs.PurposeTo describe a patient with a malignant SFT arising from the lumbar nerve root.Study designA case report and review of literature.MethodsWe describe the clinical course of the patient and the radiological and pathological findings of the tumor. The effect of systemic chemotherapy was evaluated and the relevant literature was reviewed. This work has no disclosure of funding and was approved by the Institutional Review Board of Gifu University.ResultsThe tumor had been resected previously at another hospital, but it recurred and showed multiple metastatic lesions on both lungs within 3 months. Although the patient received systemic chemotherapy, both primary and metastatic lesions were found to be stable disease according to Response Evaluation Criteria in Solid Tumors. The patient died due to cachexia 6 months after her first visit.ConclusionThis patient presented with a highly unusual tumor. Even if a tumor is a dumbbell-shaped mass, similar to a neural tumor, SFT should be considered in the differential diagnosis.     

Solitary Fibrous Tumor of the Greater Omentum,Mimicking Gastrointestinal Stromal Tumor of the Small Intestine: A Case Report

Solitary fibrous tumor (SFT) is one of the mesenchymal tumors, which rarely arises in the abdominal space. We report a very rare case of abdominal SFT, mimicking another mesenchymal tumor. A 52-year-old Japanese man was referred to our hospital for further evaluation and treatment of gallbladder polyp. Contrast-enhanced computed tomography (CT) showed an enhanced nodule within the gallbladder, and incidentally, also showed a well-circumscribed mass adjacent to the small intestine. The mass was depicted as slightly high density in plain CT, and with contrast-enhancement, the mass was partially stained in early phase and the stained area spread heterogeneously in delayed phase. Magnetic resonance imaging showed that the abdominal mass was depicted as slightly high intensity on T2-weighted imaging and low intensity on T1-weighted imaging. With double-balloon endoscopy and capsule endoscopy, we did not find any tumor inside the small intestine. These visual findings lead us to diagnose it as gastrointestinal stromal tumor of the small intestine with extraluminal growth. We planned to resect both the gallbladder polyp and the intraperitoneal tumor at the same time for pathologic diagnosis and treatment. When the operation was performed, we found a milk-white lobulated tumor on the greater omentum and the tumor was entirely resected. Microscopically, the gallbladder polyp was diagnosed as tubular adenoma, and the omental tumor was diagnosed as SFT. It is important to bear in mind that omental SFTs sometimes mimic other mesenchymal tumors and should be included in the differential diagnosis of abdominal tumor not revealed by endoscopy.Key words: Mesothelial tumor, Extrathoracic locationSolitary fibrous tumor (SFT) is one of the mesenchymal tumors, which was initially thought to usually arise in the pleura. In recent days, however, there have been several previously reported cases of extrapleural SFT; for example, orbit, thyroid, breast, and so on.¹ Nevertheless, SFTs arising in the abdominal space are still rare; in particular, there have been few reports of omental SFT in the past literature. We herein report a case of SFT of the greater omentum, which mimicked another mesenchymal tumor.     

Solitary fibrous tumor of central nervous system masquerading as meninigioma: Report of a rare case

IntroductionSolitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin that occurs most often in the visceral pleura. It rarely occurs in the central nervous system (CNS). If they occur intracranially, they are extra-axially located and develop from the meninges.Case ReportHerein, we present a case of SFT in a 45 years old female. MR imaging showed isointense to hypointense signal intensity, and marked enhancement. Microscopically, the tumors showed characteristic “patternless-pattern” architecture. Elongated tumor cells formed fascicles alternating with hypocellular densely collagenous stroma. Immunohistochemistry for STAT6, CD34, BCL2 and CD99 favours the definitive diagnosis of SFT.ConclusionIn general, complete surgical resection may offer the best chance of a favourable clinical outcome. Their biological features remain largely unknown. Hence, the clinical management and prognosis is often challenging due to the lack of comprehensive data.     

A locally aggressive solitary fibrous tumor of the leg: Case report and literature review

INTRODUCTIONThe solitary fibrous tumor (SFT) is a rare soft tissue tumor with a substantially benign clinical behavior. However, malignant neoplasms with local recurrence or distant metastases have been reported.PRESENTATION OF THE CASEThe authors present a case of an aggressive SFT of the leg, in a 55 years old Caucasian man. Radiological, histological and molecular findings are reported. The differential diagnosis, therapy and outcome of this rare tumor are also discussed.DISCUSSIONAn extensive review of literature showed SFT's clinical behavior as substantially benign, anyway aggressive or malignant neoplasms have been described. The potential risk of local recurrence and distant metastasis thus suggests wide surgical resection and careful long-term follow-up. Differential diagnosis may be quite laborious as SFT can mimic a variety of benign and malignant mesenchymal tumors; immunohistochemical analysis for CD34, CD99, vimentin and bcl-2 is then mandatory.CONCLUSIONOur clinical experience confirmed that SFT may have an aggressive behavior, however, conservative surgical treatment may be successful in the long term.     

Solitary Fibrous Tumor of the Larynx

Background True mesenchymal, non-cartilaginous neoplasms of the larynx are rare. Extrapleural solitary fibrous tumor (SFT) is a localized neoplasm characterized by proliferation of thin-walled vessels and collagen-producing cells and is considered within the “hemangiopericytoma-solitary fibrous tumor” spectrum. SFT primary in the larynx is exceptional. Design Case report set in a comparison with other cases reported in the English literature (MEDLINE 1966 to 2007). Results A 49-year old white male presented with difficulty breathing, progressive over the past 2 years. He denied dysphagia and weight loss. Past medical history was significant for asthma. He denied cigarette smoking or alcohol abuse. There were no cervical deformities on physical exam. Fiberoptic laryngoscopy was performed upon stabilization of respiratory function. A smooth, round, submucosal mass measuring 2.3 cm in greatest diameter arising from the inferior surface of left true vocal cord was causing near total obstruction of the endolaryngeal space. The mass was excised. The surface mucosa was intact and unremarkable. A cellular, spindle cell neoplasm was arranged in loose fascicles, associated with heavy collagen fiber deposition. The collagen was wiry and heavy. Cells were bland with cytoplasmic extensions. The nuclei were vesicular to hyperchromatic and elongated with inconspicuous nucleoli. Vessels were prominent and delicate, with patulous spaces. Mitotic figures were easily identified, but atypical forms were not present. The cells were strongly and diffusely immunoreactive with CD34 and bcl-2, while non-reactive with cytokeratin, EMA, actin, ALK-1, S100, desmin, and CD117. These findings confirmed a diagnosis of extraplural solitary fibrous tumor. Without further disease, the patient is alive without evidence of disease, 12 months after surgery. Conclusions The characteristic histologic pattern of solitary fibrous tumor can be noted in extrapulmonary locations. Development in the larynx is uncommon, but the tumor presents as a polypoid mass with characteristic histologic and immunophenotypic features. Conservative local excision is the treatment of choice to yield an excellent prognosis.     

A Single-Institution Experience with Eight CD117-Positive Primary Extragastrointestinal Stromal Tumors: Critical Appraisal and a Comparison with Their Gastrointestinal Counterparts

Introduction  Gastrointestinal stromal tumors (GISTs) arising from outside the gut wall also termed extragastrointestinal stromal tumors (EGISTs) are reported to be rare. Presently, their pathogenesis remains controversial, and recently, it has been proposed that EGISTs may be the result of extensive extramural growth of GISTs which lose contact with the gut wall. This study presents a single-institution experience with eight EGISTs and compares their clinicopathological features with mural GISTs in order to determine further insight to their possible origin. Methods  Between 1997 and 2008, 156 patients with pathologically proven CD117-positive primary GISTs were retrospectively reviewed. Eight tumors were identified as EGISTs, 104 were gastric GISTs, and 44 were small-bowel GISTs. Mural GISTs were classified as extramural or intra/transmural according to their gross pattern of growth. Results  There were five male and three female patients with a median age of 58 years (range, 42–81 years). All patients were symptomatic, and the tumors were located in the greater omentum (n = 2), lesser sac (n = 2), lesser omentum, retroperitoneum, small-bowel mesentery, and pancreas. The median tumor size was 140 mm (range, 55 to 220 mm). Seven of eight EGISTs were found to be in close association to the adjacent gut wall. Pathological examination demonstrated that two tumors demonstrated focal involvement of the muscularis propria of the adjacent gut wall. Four tumors demonstrated tumor abutting or adherent to the serosa but no muscle involvement and one tumor was separated from the serosa. Comparison between the clinicopathological features of EGISTs with extramural GISTs and intra/transmural GISTs demonstrated that EGISTs were significantly larger [140 range (55–220) mm vs 80 (5–260) mm vs 50 (15–190) mm, P = 0.049, P < 0.001 respectively]. Conclusion  The occurrence of true EGISTs is rare. Most cases demonstrate some form of communication or contact with the gut wall, and EGISTs are significantly larger than extramural or intra/transmural GIST. These observations suggest that most, if not all, cases of EGISTs are likely to represent mural GISTs with extensive extramural growth with eventual loss of contact with the muscle layer of the gut.     

Mediastinal solitary fibrous tumor with right diaphragm invasion: Report of a case

Mediastinal solitary fibrous tumors (SFTs) are rarely found in adults and there are few reports describing primary mediastinal SFT invading the diaphragm. We report the case of a 47-year-old woman with a large right inferior mediastinal SFT. Magnetic resonance imaging showed the tumor invading the right lower lobe of the lung and the right hemidiaphragm, with displacement of the inferior vena cava (IVC) and right lobe of the liver. Angiogram showed IVC stenosis. To our knowledge, this is the first report of complete resection of the tumor combined with right lower lobectomy of the lung and partial resection and reconstruction of the right diaphragm with a Dacron flap.     

Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up

Background  

Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT.     

Kimura disease: a brief clinical report

Kimura disease (KD) is an uncommon idiopathic inflammatory disease (Khoo and Chan, Cutis 70(1):57–61, 2002). This chronic inflammatory soft tissue disease is characterized by lymphoid and angiomatous tissue proliferation with lymphadenopathy, eosinophilia, and elevated serum IgE levels (Khoo and Chan, Cutis 70(1):57–61, 2002). Lesions are usually located on the head and neck area (Kung et al., Pathology 16:39–44, 1984), and the disease is more common among Asians. In this study, we present a patient with a diffuse mass on his left side of the face from the parotid area to the temporal fossa, who is also suffering from nephrotic syndrome. A review of the literature is presented.     

Morphological Distribution of Metastatic Foci in Sentinel Lymph Nodes with Gastric Cancer

Background The TNM classification defines micrometastasis (MM) and isolated tumor cells (ITC) in lymph nodes (LN). Sentinel node (SN) navigation surgery has been introduced in gastrointestinal cancer. Few reports have examined the morphological distribution of MM and ITC of SN in gastric cancer. The purpose of this study was to clarify the clinical significance of the morphological distribution of cancer cells in SNs according to metastasis (MA), MM, and ITC. Methods All dissected LNs obtained from 160 consecutive patients with mapped SNs arising from cT1–2 N0 tumors were examined. Metastasis in these LNs was examined by histology and cytokeratin staining. The distribution of MA, MM, and ITC was classified as marginal sinus (MS), intermediate sinus (IS), parenchymal (PA), and diffuse types (DF). Results Nodal metastases were detected in 65 SNs from 30 patients and MA, MM, and ITC accounted for 53.9%, 21.5%, and 24.6%, respectively. MS, IS, PA, and DF accounted for 57%, 6%, 17%, and 20.0%, respectively. Patients with metastasis of non-MS had more nodal metastasis in non-SNs (P = .025) and had nodal metastasis in second tier (P = .009), compared with the patients with metastasis of MS. The incidence of metastasis in non-MS was higher in tumors larger than 40 mm than those smaller than 40 mm (P = .011). Conclusion When performing SN navigation surgery in gastric cancer, we should keep in mind that the patients with tumor larger than 40 mm in size and nodal metastasis of non-MS may have non-SN metastasis and nodal metastasis in second tier.     

Surgical Therapy of Pancreatic Pseudocysts

Background  Pancreatic pseudocysts are a common complication associated with acute and chronic pancreatitis. Fifteen percent and 40% of patients diagnosed with either acute or chronic pancreatitis, respectively, develop pseudocysts (Grace and Williamson, Br J Surg, 80:573–581, 1993). The treatment of pancreatic pseudocysts has evolved since the early 1980s, and changes in management have lead to an improved understanding of the pathophysiology of pseudocysts as well as necessary treatment paradigms. Conclusions  It has become evident that not all pseudocysts are equal. Pseudocysts arising in the setting of acute pancreatitis have a different pathophysiologic basis than those arising from chronic pancreatitis. Moreover, even those pseudocysts that arise in acute pancreatitis exhibit unique features. Pseudocysts that develop from a mild episode of pancreatitis, complicated by pancreatic duct disruption, differ significantly from those developed as a consequence of severe acute necrotizing pancreatitis with severe distortion of the pancreatic parenchyma or pancreatic duct. This review will focus on the surgical therapy of pancreatic pseudocysts in the context of the underlying pathophysiology and alternative nonoperative therapies.     

Solitary fibrous tumor of the breast: A case report and the review of the literature

Solitary fibrous tumors (SFT) are rare mesenchymal neoplasms. They were first described as spindle‐cell tumors originating from the pleura, but they may arise in any anatomical site. SFT of the breast is an exceedingly rare clinical entity. Our literature review yielded only 21 cases reported so far. We describe a case of a SFT of the breast in a premenopausal patient who presented with a gradually enlarging palpable breast mass. Diagnostic evaluation and management are discussed along with a review of the relevant literature. There are no pathognomonic imaging findings of SFT of the breast. Complete surgical resection with clear margins is the gold standard of treatment. Thorough immunohistochemical analysis is crucial to obtain a definitive diagnosis. Although most SFTs run an indolent course, in some cases the clinical behavior can be unpredictable and a long‐term follow‐up for all patients is therefore mandatory.     

Clinicopathological findings in a case series of extrathoracic solitary fibrous tumors of soft tissues

Background  

Solitary fibrous tumors (SFT) represent a rare entity of soft tissue tumors. Previously considered being of serosal origin and solely limited to the pleural cavity the tumor has been described in other locations, most particularly the head and neck. Extrathoracic SFT in the soft tissues of the trunk and the extremities are very rare. Nine cases of this rare tumor entity are described in the course of this article with respect to clinicopathological data, follow-up and treatment results.     

A massive hepatic tumor demonstrating hepatocellular,cholangiocarcinoma and neuroendocrine lineages: A case report and review of the literature

IntroductionMixed hepatocellular and cholangiocarcinoma tumors (MHCC) are described in the literature, as are the more rare mixed adenoneuroendocrine carcinomas (MANC) of hepatobiliary origin. Only two cases of tumors with characteristics of all three histologies/phenotypes have been previously described in one Chinese study.Presentation of caseHerein we report clinical, microscopic and molecular features of a 25 cm mixed hepatic tumor with hepatocellular, cholangiocarcinoma and neuroendocrine differentiation arising in an otherwise healthy 19-year-old North American Caucasian male without any identifiable risk factors.DiscussionThe patient underwent multimodality imaging and the tumor was biopsied preoperatively, and it was initially interpreted to be hepatocellular carcinoma fibrolamellar type. A left trisegmentectomy with lymphadenectomy was performed and the tumor was definitively diagnosed based on the surgically resected specimen. Integrated microscopic and molecular features defined the differing biological aggressiveness of growth pattern components. Cases in the literature of MHCC and rare cases of MANC have largely undergone aggressive surgical resection as well, however the majority of studies on mixed hepatic tumors to date reflect Eastern patient cohorts and populations with underlying liver disease, thereby limiting extrapolation on management or outcomes in this case.ConclusionThis is one of the only reports of a hepatic tumor arising from hepatocellular carcinoma, cholangiocarcinoma and neuroendocrine lineages. Increased awareness of this tumor type may optimize improve future management.     

Post auricular dermoid cyst��a rare lump behind the ear

Dermoid cysts are benign developmental anomalies. They are postulated to originate from the congenital inclusion of germ layers in the deeper tissues along the lines of embryonic fusion (New and Erich Surg, Gynecol Obstet 65:48–55, 1937). Dermoid cysts can be divided into three types according to their histological characteristics, namely epidermoid, dermoid, and teratoid (Tuffin and Theaker, Int J Oral Maxillofac Surg 20:275, 1991). The majority of dermoid cysts arise in the ovaries. Those occurring in the cervicofacial region are uncommon, accounting for about 7% of all dermoids (De Souza et al., Plast Reconstr Surg 112(7):1972, 2003). Dermoid cysts around the auricular region are rare. To the best of our knowledge, there have been only four cases of post-auricular dermoid cyst reported in literature (Bauer et al., Arch Dermatol 130:913, 1994; De Souza et al., Plast Reconstr Surg 112(7):1972, 2003; Meagher and Morrison, Br J Plast Surg 54:336, 2001; Samper, Plast Reconstr Surg 106:947, 2000. We hereby report the fifth case of this rare condition.     

Wide local excision for anal GIST: A case report and review of literature

IntroductionGastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin commonly detected in stomach and small bowel. GIST arising primarily from the anal canal is extremely rare. Due to the malignant potential, these tumors are treated with radical surgery like abdominoperineal resection. But with the advent of imatinib therapy and a better understanding of the tumor biology, some cases have been successfully treated with wide local excision.Presentation of caseWe describe a case of a 70-year-old lady presenting with a 2 cm mass in the anal canal. Endoanal ultrasound revealed a well-circumscribed solid nodule in the intersphincteric space. The patient was successfully treated by wide local excision and adjuvant therapy with imatinib mesylate.DiscussionOnly 14 confirmed cases of primary anal GIST have been reported in the literature. It appears as a well circumscribed hypoechoic mass arising from the intersphincteric space encroaching into the lumen on endorectal ultrasound. Lymphadenopathy is absent. Anal sphincters get involved as the lesion increases in size. Treatment is often planned based on the extent of the disease, the mitotic rate, patient’s general condition and willingness for a permanent colostomy.ConclusionSmall lesions (<2 cm) with low mitotic rate may be successfully managed by local excision. Radical surgery should be reserved for large, aggressive tumors.     

Advantages of the laparoscopic approach for intersphincteric resection

Background  

Intersphincteric resection (IRS) is a surgical technique used to preserve sphincter function, mainly cases of low rectal cancer located less than 5 cm from the anal verge [1, 2]. There have been reports of laparoscopic ISR [3, 4], but discussion of the specific techniques used in this laparoscopic surgical procedure have not been sufficient. For better outcomes of this sophisticated procedure, extreme care must taken to prevent perforation of the rectal wall and to preserve the external sphincter muscle. The most difficult steps for ISR are the circular dissection and separation of the internal sphincter muscle from the external sphincter and puborectalis using the perineal approach. The authors’ techniques and the advantages of laparoscopic ISR are shown by a video presentation of three rectal tumor cases. Also, the perioperative outcomes for the patients who underwent laparoscopic ISR with this technique are described.     

Hemangiopericytoma/solitary fibrous tumor of pectoralis major muscle mimicking a breast mass

INTRODUCTIONHemangiopericytoma (HPC)/solitary fibrous tumor (SFT) is a very uncommon tumor of uncertain malignant behavior. In 1942, Stout and Murray first characterized these neoplasms as “vascular tumors arising from Zimmerman's pericytes” and till now hemangiopericytomas and solitary fibrous tumors of the soft tissues are regarded as features of the same entity in the soft tissue fascicle.PRESENTATION OF CASEWe present a case of hemangiopericytoma/solitary fibrous tumor of the pectoralis major muscle in a 33-year-old female. She first noticed a painless mass in her right breast. Ultrasound of the breast revealed a large heterogeneously hypoechoic lesion within the pectoralis major muscle. Fine needle aspiration of the tumor did not produce any meaningful result. The lesion was completely removed by surgical resection. Histologically, the tumor had staghorn-like vasculature and immunohistochemistry for CD34 was positive, whereas desmin, smooth-muscle actin, S-100 protein, cytokeratins (AE1/AE3) and epithelial membrane antigen (EMA) were all negative. A diagnosis of hemangiopericytoma/solitary fibrous tumor was rendered.DISCUSSIONTumors comprising the HPC/SFT spectrum represent a small subset of soft tissue sarcomas and are found virtually at any site in the body. Wide surgical resection can achieve favorable long-term survival.CONCLUSIONDue to the rarity and unpredictable biological potential of these tumors, long-term follow-up is mandatory even after radical resection, because recurrence or development of metastasis may be delayed many years.     

Malignant peripheral nerve sheath tumor of kidney—A case report

INTRODUCTIONMalignant peripheral nerve sheath tumors (MPNSTs) are very rare soft-tissue tumors. These tumors originate from Schwann cells or pluripotent perineural cells of the neural crest. An isolated MPNST of the kidney is extremely rare and has been reported in only six cases to date.PRESENTATION OF CASEA 30-year-old female with an unremarkable past medical and surgical history presented with an enlarging, right-sided abdominal mass for the last 3 months. A CT scan of the abdomen and pelvis showed a large exophytic mass arising from the right kidney. A metastatic work-up was negative, so a right radical nephrectomy was performed. Histopathological and immunohistochemical studies confirmed the diagnosis of an MPNST.DISCUSSIONMPNSTs are rare and aggressive soft-tissue sarcomas that are usually associated with neurofibromatosis type 1 (NF-1) but that also occur post-radiation and sporadically. These tumors are highly malignant neoplasms with a high recurrence rate and distant metastases. Complete surgical excision remains the mainstay of treatment due to the limited response of MPNSTs to both chemotherapy and radiotherapy.CONCLUSIONMPNSTs arising from the kidney are very rare. Complete surgical resection provides the only hope for cure. Postoperative chemotherapy and radiotherapy are still controversial, although postoperative radiotherapy is currently recommended by an oncology consensus group.     

Is undergraduate physiotherapy study a risk factor for low back pain? A prevalence study of LBP in physiotherapy students

Background  

Following reports of high prevalence of low back pain (LBP) in young physiotherapists [17–22], we investigated whether LBP was a problem for undergraduate physiotherapy students.