Primary Mesenchymal Tumors Research Articles

Vena Cava ınvasion by Adrenal Leiomyosarcoma

Primary adrenal mesenchymal tumors are extremely rare. These tumors are hard to diagnose, and similar to certain adrenal tumors, as they do not produce hormones, and they can only manifest themselves when the tumor reaches an advanced size. These tumors are generally detected incidentally. This study reports a rare case of primary leiomyosarcoma of the right adrenal gland with vena cava invasion, in a 70-year-old woman who presented with right flank pain. Computerized tomography showed an adrenal mass with a diameter of 78 mm, which exerted pressure on the vena cava inferior. The invasive part was excised by using adrenalectomy and cavatomy. Tumor invasion was determined on the wall of the vena cava. Histopathological examination on 10× magnification showed 8-10 mitotic events. Immunohistochemical staining showed that the cells were SMA (+), desmin (+), cytokeratin (-), and Bcl-2 (-). The Ki67 proliferation index was 70%. Widespread metastasis developed six months after the adrenalectomy.

The changing face of GIST: implications for pathologists

Gastrointestinal stromal tumour (GIST) is now recognised as the most common primary mesenchymal tumour of the gut. A number of different parameters have been identified to aid prediction of clinical behaviour, but prognostication for an individual remains difficult. The pathologist plays a crucial role in guiding management of these tumours, but is faced with a number of challenges in so doing. This review describes the variable pathological features that may be encountered, and examines some of the issues in the pathology reporting of GIST and attempts to provide some guidance in factors that should be addressed in a comprehensive pathology report.

Primary intrathoracic malignant mesenchymal tumours: computed tomography features of a rare group of chest neoplasms

ObjectivesTo describe the computed tomography (CT) features in a case series of primary intrathoracic extracardiac malignant mesenchymal tumours (sarcomas).MethodsA 5-year retrospective research was conducted, and 18 patients were selected. CT exams were reviewed by two chest radiologists, blinded to tumour pathological type, origin and grade. Lesions were described in relation to location, size, shape, margins, enhancement, presence of cavitation, calcifications, ground glass component, intratumoural enhanced vessels, pleural effusion, pleural tags, lymphangitis, chest wall/rib involvement and pathological lymph nodes.ResultsThe readers described five pulmonary, six mediastinal and seven pleural/wall based lesions. Mean largest diameter was 103 mm. The most frequent shape was irregular (n = 12), most predominant margin was smooth (n = 12) and enhancement was mostly heterogeneous (n = 8). Intratumoural vessels and pleural effusion were seen in 11 patients. Pathological lymph nodes were present in four cases and calcifications in two cases.ConclusionsSome frequent radiological features were described independently of tumour location and subtype. A sarcoma should be included as a major differential diagnosis when the radiologist faces an intrathoracic mass of large size (>70 mm) but with well defined smooth or lobulated margins, especially if presenting intratumoural vessels, associated pleural effusion but no significant lymphadenopathy.Main messages• Malignant mesenchymal tumours (sarcomas) are rare and can arise from any structure in the chest.• Intrathoracic sarcomas show some frequent radiological features, independent of location and type.• Some CT features may help the radiologist suspect for a sarcoma instead of other more common tumours.

Primary Malignant Mesenchymal Tumors of the Liver

Primary malignant mesenchymal tumors represent a rare subset of hepatic lesions. These lesions pose unique diagnostic challenges for a pathologist because they are infrequently seen in general practice and can be easily mistaken for their benign counterparts or mimickers. In the current era of personalized medicine, the precise pathological diagnosis of these lesions is critically important to guide targeted therapy. In this article, we first briefly review the key clinical, pathological, immunohistochemical, and molecular characteristics of both common and uncommon primary malignant mesenchymal tumors of the liver. We then outline a practical diagnostic approach based on the unique features of each entity. This approach may help the general pathologist and pathology trainees navigate through these complex uncommon lesions and arrive at a specific diagnosis.

Primary mesenchymal tumors of the liver: A practical diagnostic approach

Primary mesenchymal tumors represent a rare subset of hepatic lesions with overlapping spindle cell morphology. These lesions pose unique diagnostic challenges for pathologists as they are infrequently seen in general practice and can be easily mistaken for their benign counterparts or mimickers. In the current era of personalized medicine, precise pathologic diagnosis of these lesions is critically important to guide targeted therapy. This expectation is difficult to meet when pathologists face uncommon lesions outside their scope of experience. This challenge is further exacerbated when interpreting small biopsy samples. In this lecture, we first present the clinicopathological features of 6 typical cases: inflammatory pseudotumor, angiomyolipoma, epitheleioid hemagioendothelioma, malignant fibrous histiocytoma, leiomyosarcoma, and follicular dendritic cell sarcoma. On each case, the focus of discussion is on the morphology characteristics and how to differentiate them from metastatic tumors, non-neoplastic lesions and other types of primary spindle cell neoplasms. We then outline a practical diagnostic approach based on the unique features of each entity. This lecture provides a comprehensive guidance to general pathologists and pathology trainees on how to navigate through this group of seemingly complex entities and to arrive at a specific diagnosis.

Primary mesenchymal tumor of adrenal

Primary mesenchymal tumor of adrenal is very rare. We present a case of adrenal mesenchymal tumor. A 48-year-old Chinese woman was admitted to hospital presenting symptom of distension and pain at the left upper quadrant of the abdomen for one year. Computed tomography images showed a circumscribed mass at the left adrenal region which measured about 9 × 12cm. Macroscopy, a nodular mass in the left adrenal gland with an capsule was 13 × 10 × 8cm in size, and the cut surface of which was soft, grayish white and yellow, with cystic change and hemorrhage in some areas, a little cortex at the periphery remained. Histopathology, compact short spindle, oval and polygonal tumour cells arranged in sheets, partly, in haemangiopericytomatous pattern. The tumour cells were uniform with ovoid pale-staining nuclei and inconspicucous nucleoli. Cytoplasm was sparse and cell borders were indistinct. Mitotic figures were about 2–3/10 HPF.Focally, necrosis and degeneration could be found. Immunohistochemical staining revealed the tumor cells were positive for CD117, vimentin, CD99, Bcl-2 and EMA. While Keratin, Dog-1, CD34, α-inhibin and calretinin <i>et al</i>. were negative. The patient is survival now 4.5 years after adrenalectomy without any therapy, but there has been a local recurrence since last year.

Synchronous gastric gastrointestinal stromal tumor and colon adenocarcinoma: a case report.

Gastrointestinal stromal tumors (GISTs) represent the majority of primary mesenchymal tumors of the gastrointestinal tract. They are generally considered to be solitary tumors and therefore the synchronous occurrence with other primary malignancies of gastrointestinal track is considered a rare event. Here we present the case of a 75-year-old man admitted to our hospital with a 10-day history of gastrointestinal bleeding. Colonoscopy revealed an ulcerative mass of 4 cm in diameter in the ascending colon. Gastroscopy revealed a bulge in the gastric body measuring 1 cm in diameter with normal overlying mucosa. Surgical intervention was suggested and ileohemicolectomy with regional lymph node resection along with gastric wedge resection was performed. Pathologic examination of the ascending colon mass showed an invasive moderately differentiated adenocarcinoma stage III B (T3N1M0). Grossly resected wedge of stomach showed a well circumscribed intramural tumor which microscopically was consistent with essentially benign gastrointestinal stromal tumor (according to Miettinen criteria). The patient did not receive additional treatment. Two years later the patient showed no evidence of recurrence or metastasis.

Tumor‐Induced Osteomalacia Resulting from Primary Cutaneous Phosphaturic Mesenchymal Tumor: A Case and Review of the Medical Literature

Journal of Cutaneous PathologyVolume 40, Issue 9 p. 780-784 Cover Quizlet Tumor-Induced Osteomalacia Resulting from Primary Cutaneous Phosphaturic Mesenchymal Tumor: A Case and Review of the Medical Literature Kevin H. Gardner, Kevin H. Gardner Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorWonwoo Shon, Wonwoo Shon Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorAndrew L. Folpe, Andrew L. Folpe Division of Anatomic Pathology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorCarilyn N. Wieland, Carilyn N. Wieland Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorPeter J. Tebben, Peter J. Tebben Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorChristian L. Baum, Corresponding Author Christian L. Baum Department of Dermatology, Mayo Clinic, Rochester, Minnesota Correspondence to: e-mail: [email protected]Search for more papers by this author Kevin H. Gardner, Kevin H. Gardner Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorWonwoo Shon, Wonwoo Shon Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorAndrew L. Folpe, Andrew L. Folpe Division of Anatomic Pathology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorCarilyn N. Wieland, Carilyn N. Wieland Department of Dermatology, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorPeter J. Tebben, Peter J. Tebben Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MinnesotaSearch for more papers by this authorChristian L. Baum, Corresponding Author Christian L. Baum Department of Dermatology, Mayo Clinic, Rochester, Minnesota Correspondence to: e-mail: [email protected]Search for more papers by this author First published: 21 August 2013 https://doi.org/10.1111/cup.12209Citations: 16Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Citing Literature Volume40, Issue9September 2013Pages 780-784 RelatedInformation

Sporadic Congenital Hyperplasia of Interstitial Cell of Cajal of Rectum as a Cause of Chronic Constipation

Gastrointestinal stromal tumors (GIST) are the usual primary mesenchymal neoplasms of the GI tract. Interstitial cells of Cajal (ICCs) are intestinal pacemaker cells that instigate peristalsis in the stomach and intestine, and are measured to be precursors of GIST. We are reporting a 17 year old boy with chronic constipation since childhood and a huge rectum in abdominal radiography, who underwent exploratory laparotomy, resection of huge rectum and construction of sigmoid colostomy. Pathology report was Hyperplasia of Interstitial Cells of Cajal.

Spindle Cell Lipoma of the Larynx

Among the primary mesenchymal tumors of the hypopharynx and larynx, lipomas are rare. Macroscopically, they often resemble a retention cyst or laryngeal nodule. Spindle cell lipomas (SCLs) are an uncommon variant of lipoma. SCLs are extremely rare in the larynx; as far as we know, only 4 cases have been previously described in the literature. We present a new case of laryngeal SCL in a 65-year-old man who presented with a 1-year history of hoarseness, choking spells, stridor, and dyspnea. Examination revealed the presence of a large polyp on the left true vocal fold that had caused stenosis of the posterior glottis. The polyp was removed endoscopically, and the patient's stridor and dyspnea resolved. Histologically, the tumor was composed of bland, CD34-positive spindle cells with an abundant fibrous and myxoid stroma interspersed with mature fatty tissue. The patient was free of local recurrence at 2 years of follow-up.

Intranodal Palisaded Myofibroblastoma

Intranodal palisaded myofibroblastoma is a rare benign primary mesenchymal neoplasm originating from differentiated smooth muscle cells and myofibroblasts. The precise etiology and pathogenesis has not been adequately explained as yet. Very few series and cases have been reported in the literature. Though inguinal region is the commonest site of this rare tumor, but the tumor at other diverse sites have been reported. Because of its rarity, it can be often misdiagnosed and confused with other disorders and more commonly with metastasis. We report an extensive review of literature about intranodal palisaded myofibroblastoma--its characteristics, presentations, features, and management.

Primary osteosarcoma of the thyroid gland: report of a rare neoplasm

Primary mesenchymal tumors of the thyroid gland are extremely rare. The authors report a case of primary thyroid osteosarcoma in a male patient presenting a tumoral mass in the neck. CT scan demonstrated a large tumor in the right thyroid lobe with areas of calcification. The surgical specimen consisted of a hard brown-gray tumor, measuring 13 × 11 × 7.5 cm. Microscopy revealed a high-grade malignant neoplasm composed of polygonal cells of intermediate size, chondroid pattern in some areas and osteoid formation. The process was immunonegative for AE1/AE3, glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), p53 and thyroid transcription factor-1 (TTF-1), and immunopositive for CD99 and S100. Thus the diagnosis of primary osteosarcoma of the thyroid gland was established.

Primary adrenal leiomyosarcoma: A case report with immunohistochemical study and review of literature

Primary adrenal mesenchymal tumors are exceptionally rare. Diagnosis is based entirely on histological and immunohistochemical evaluation which is indispensable not only for determining tumor type but also for predicting biological behavior. We report a rare case of primary leiomyosarcoma of the left adrenal gland, in a 60 year old woman who presented with flank pain. Computed tomography revealed a well defined left adrenal tumor which was surgically resected. Histological examination of the tumor showed malignant spindle cells in interlacing fascicles and whorls. Nuclear pleomorphism, tumor giant cells and abnormal mitotic figures were seen. On immunohistochemistry, the tumor cells showed reactivity for smooth muscle actin, vimentin and desmin; and were negative for cytokeratin, S100 protein, CD117 and HMB-45. A diagnosis of primary adrenal leiomyosarcoma was offered. Postoperative recovery of the patient was uneventful and the patient was symptom free with no evidence of tumor metastasis or recurrence 21 months after surgery.

An Unusual Case of Primary Hepatic Leiomyosarcoma

Purpose: Primary hepatic leiomyosarcoma is exceedingly rare and only few cases have been reported in literature. Presentation is usually nonspecific and diagnosis is often delayed until tumors reach a large size. This leads to a dismal prognosis. The tumors are not yet fully understood hence standard of care not yet well defined. Case Presentation: A 51-year-old male patient presented to the hospital with intractable hiccups, poor oral intake and 20 pounds weight loss. He denied abdominal pain, nausea, vomiting or irregular bowel habits. His comorbidities included seizure disorder and hypertension. He had no history of liver disease or alcohol abuse. On physical examination he appeared cachectic. Abdominal examination revealed a hepatomegaly of about 6cm below the right costal margin. Laboratory analysis revealed alanine aminotransferase 81 IU/L (0-34 IU/L), aspartate aminotransferase 56 IU/L (0-40 IU/L) and alkaline phosphatase 160 IU/L (28 -94 IU/L), serum albumin 2.8 g/dl (3.4 -5 g/dl). Serum bilirubin prothrombin time, white blood cells, platelets and α-fetoprotein were normal. Antibody to hepatitis C and hepatitis B surface antigen were negative. Abdominal computed tomography showed a hypodense rim enhancing 3.5 cm by 2.5 cm lesion with a necrotic center within segment VI of the liver. A CT guided biopsy of the lesion revealed a tumor comprised of spindle cells arranged in interlacing pattern. There was minimal nuclear pleomorphism, tumor necrosis and no increase in mitotic activity. Immunomarker stains were positive for smooth muscle actin but negative for desmin, CD117 and CD34. These pathological features were consistent with a diagnosis of spindle cell neoplasm, low grade leiomyosarcoma involving the liver. Discussion: Hepatic tumor of mesenchymal origin is rare, accounting for only 1.2% of all primary hepatic tumors. The most common type of primary malignant mesenchymal tumor is angiosarcoma (36%) followed by leiomyosarcoma, fibrosarcoma and unspecified sarcoma. Most of hepatic leimyosacoma are metastatic from other sites of leiomyosarcoma including gastrointestinal tract, uterus, retroperitoneum and lung. Liver function tests are usually variable while carcinoembryonic antigen and α-fetoprotein are normal. Cut surface of the liver is often pinkish-white with yellow areas of necrosis or dark red hemorrhagic foci. Mean survival of untreated hepatic leiomyosarcoma is dismal (about 10 months). Surgical excision appears to prolong survival however chemotherapy is the alternative therapy for inoperable patients. In conclusion we present a rare case of primary hepatic leiomyosarcoma. Prognosis is poor and clinical diagnosis is challenging in cases of small tumors.

LY294002 Induces G0/G1 Cell Cycle Arrest and Apoptosis of Cancer Stem-like Cells from Human Osteosarcoma Via Down-regulation of PI3K Activity

Osteosarcoma, the most common primary mesenchymal malignant tumor, usually has bad prognosis in man, with cancer stem-like cells (CSCs) considered to play a critical role in tumorigenesis and drug-resistance. It is known that phosphatidylinositol 3-kinase (PI3K) is involved in regulation of tumor cell fates, such as proliferation, cell cycling, survival and apoptosis. Whether and how PI3K and inhibitors might cooperate in human osteosarcoma CSCs is still unknown. We therefore evaluated the effects of LY294002, a PI3K inhibitor, on the cell cycle and apoptosis of osteosarcoma CSCs in vitro. LY294002 prevented phosphorylation of protein kinase B (PKB/Akt) by inhibition of PI3K phosphorylation activity, thereby inducing G0/G1 cell cycle arrest and apoptosis in osteosarcoma CSCs. Further studies also demonstrated that apoptosis induction by LY294002 is accompanied by activation of caspase-9, caspase-3 and PARP, which are involved in the mitochondrial apoptosis pathway. Therefore, our results indicate PI3K inhibitors may represent a potential strategy for managing human osteosarcoma via affecting CSCs.

A Rare Case of Hilar Lymph Node Schwannoma

Schwannoma arising in a lymph node is an extremely rare primary mesenchymal tumor of the lymph node. Although intranodal schwannoma is benign, this lesion in a lymph node may be initially alarming and it may suggest a diagnosis of intranodal metastasis from a spindle cell tumor. Microscopic examination of tumor shows Antoni A areas, which are moderate to highly cellular areas of elongated cells arranged in fascicles, and Antoni B areas, which are hypocellular areas with myxoid and microcystic change. Tumor cell morphology is similar in both areas with elongated shape and regular oval nuclei. Immunohistochemistry report shows strong and diffuse positivity for S-100 which is consistent with Schwann cell origin. We report a rare case of intranodal schwannoma arising in the hilar lymph node with its clinical, light microscopic, and immunohistochemical findings.

Oral metastasis of chondroblastic osteosarcoma

Osteosarcoma is the most common primary malignant mesenchymal tumor, accounting for approximately 20% of sarcomas, with 5% incidence in the jaws. They present various clinical and histological aspects as well as variable disease prognosis and outcome. About 50% of all osteosarcomas are osteoblastic, 25% fibroblastic, 25% chondroblastic. Metastasis of osteosarcoma in the oral cavity is rare, and very few cases have been described so far in the literature. This article presents a metastatic case of chondroblastic osteosarcoma in the mandibular right-attached gingiva arising from 4th rib. This case report further suggests that chondroblastic osteosarcoma has poor prognosis.

Histopathological, Immunohistochemical and Ultrastructural Studies of a Renal Mesenchymal Tumor in a Young Beagle Dog

A 15-month-old male beagle dog used in a toxicity study had a primary renal mesenchymal tumor. Macroscopically, the tumor was a gray-white mass which was found in the right kidney, and extended from the capsule to a position slightly compressing the medulla. Microscopically, most of the tumor cells showed a myxoid pattern, in which the matrix was positive for alcian blue staining. In the other parts of the tumor, a fascicular and wavy pattern was observed, and the matrix was full of collagen fibrils. Immunohistochemically, tumor cells were positive for vimentin and fibronectin, and negative for cytokeratin, desmin, α-smooth muscle actin, Von Willebrand factor, cyclooxigenase-2 and myelin basic protein. As a result, we diagnosed this case to be a renal mesenchymal tumor. Based on the microscopic findings, interstitial characteristics and immunohistochemical features, the present case was classified as a congenital mesoblastic tumor.

Intranodal palisaded myofibroblastoma originating from retroperitoneum: an unusual origin

BackgroundIntranodal palisaded myofibroblastoma is one of the primary mesenchymal tumours. The inguinal region is the commonest site of this rare tumour. As there are only about 55 such cases reported in the literature, the precise aetiology and pathogenesis have yet to be explained adequately. Here we report a case of a 72 year old man presented with incidental finding of intranodal palisaded myofibroblastoma in the retroperitoneal region.Case PresentationA 72-year old man presented with abdominal pain in right upper quadrant with an incidental finding of abdominal mass in the right flank. The computerised tomogram scan of abdomen confirmed acute cholecystitis with a 5 x 5 cm retroperitoneal mass. He underwent cholecystectomy with excision of this mass. He recovered well following his operation and was discharged from the hospital. Histological examination confirmed the diagnosis of intranodal palisaded myofibroblastoma.ConclusionTo our knowledge, this is the first case of intranodal palisaded myofibroblastoma originating from retroperitoneum. Along with the rarity of this case, we also discussed its typical histopathological findings, aetiology and pathogenesis.

Primary lung sarcoma: A review of 25 cases in a single tertiary institution.

e20528 Background: Primary lung mesenchymal tumours are quite rare occurring in <1% of all lung cancers (LC). Data is limited regarding the pathophysiology, natural history and treatment of these heterogeneous neoplasms. Treatment options are based on experience from other primary sarcomas. Methods: Medical records of 2,560 patients (pts) diagnosed with LC between 2003-2011 were screened. We examined pts’ demographics, clinical data and treatment details Results: 25/2,560 pts (1%) were diagnosed with primary mesenchymal lung tumours. Male:female ratio was 17:8 and median age 63 years (range 21-81). The histological subtypes as reported were: synovial sarcoma (n=9), Ewing’s Sarcoma/PNET (n=3), sarcomatous carcinoma (n=3), mesenchymal tumours/sarcoma (n=3), fibrosarcoma (n=2), malignant fibrous histiocytoma (n=1), leiomyosarcoma (n=1), blastoma (n=1), biphasic sarcoma (n=1) and polymorphic sarcoma (n=1). Immunocytochemistry showed overexpression of vimentin in 23 pts and of CD99 in 8 pts. Commonest presenting symptoms were chest pain (n=11) and dyspnoea (n=10). Performance Status was satisfactory (ECOG 0-1) in most pts (n=17). Smoking history was reported by 17/25 pts. 15 pts presented with advanced disease and were treated with chemotherapy (CT) (group A). 10 pts with initially localised disease (group B) were treated with either surgery (n=8) or radical chemoradiotherapy (CRT) (n=2). 4 pts received adjuvant CT. The commonest first line regimens were CAV (Cyclophosphamide, Doxorubicin, Vincristine) (n=10, 4pts with SD as best response) and platinum based combinations (n=7, best response: 1 PR, 3 SD). Median overall survival (OS) by Kaplan-Meier estimator for group A and group B were 6.5 months (95% CI 0.0-16.2) and 26 months (95% CI 23.8-28.2) respectively, with 2 pts of group A still on CT. Conclusions: Primary lung sarcoma is rare, occurring in approximately 1% of primary LC. OS in advanced disease is limited to less than 1 year. Surgery and radical CRT, when appropriate, are associated with better survival. Platinum agents seem active and warrant further prospective evaluation in this disease.