Round Tumor Research Articles

Unexpected ectopic thyroid tissue in the orbit: a clinical case report

AbstractPurpose To present clinical case report of the benign ectopic thyroid tissue in the orbit.Methods 75‐year‐old woman with a slowly growing, painless, elastic tumor in the left inferior –lateral part of the orbit complained about diplopia, eye globe protrusion and dislocation medialy‐superiorly. Symptoms were progressing for two years. Head magnetic resonance imaging (MRI), tumor excision and histopathological examination of the obtained specimen were performed.Results Head MRI showed 18x14 mm in size, non‐homogenic, contrast accumulating mass with well defined margins in the inferior –lateral part of the orbit. Gross examination of the excised mass revealed grey color, round tumor, covered by connective tissue. Histologicaly tumor was well circumscribed, composed of micro‐/ macrofolicules with colloid. Folicular epithelium had no atypical changes. These criteria support diagnosis of ectopic thyroid tissue with slight signs of goiter. In the National Pathology Registre it was found that one year before patient had thyroidectomy and histological diagnosis of nodular thyroid hyperplasia and follicular adenoma was established. Possibility of follicular carcinoma metastasis to the orbit was rejected reviewing both cases by three pathologists and only usual Hematoxylin/Eosin method was used for staining.Conclusion There are rear case reports of ectopic thyroid tissue. To our knowledge this is the first description of thyroid tissue with signs of goiter in the orbit.

Three cases of retroperitoneal schwannoma diagnosed by EUS-FNA

Schwannomas are peripheral nerve tumors that are typically solitary and benign. Their diagnosis is largely based on surgically resected specimens. Recently, a number of case reports have indicated that retroperitoneal schwannomas could be diagnosed with endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). We report the diagnosis of three cases of schwannoma using EUS-FNA. Subjects were two males and one female, ages 22, 40, and 46 years, respectively, all of whom were symptom-free. Imaging findings showed well-circumscribed round tumors. However, as the tumors could not be diagnosed using these findings alone, EUS-FNA was performed. Hematoxylin-eosin staining of the resulting tissue fragments revealed bland spindle cells with nuclear palisading. There was no disparity in nuclear sizes. Immunostaining revealed S-100 protein positivity and all cases were diagnosed as schwannomas. Ki-67 indexes were 3%-15%, 2%-3%, and 3%, respectively. No case showed any signs of malignancy. As most schwannomas are benign tumors and seldom become malignant, we observed these patients without therapy. All tumors demonstrated no enlargement and no change in characteristics. Schwannomas are almost always benign and can be observed following diagnosis by EUS-FNA.

Editorial: Brain tumors

For over a century physicians have tried to use what they see under the microscope to predict the behavior of brain tumors and to determine which treatment strategies would best benefit their patients. This approach has been particularly frustrating in pediatric neurooncology. What appears to be the same tumor in an infant may behave quite differently in an older child. That “same tumor” may have a very different prognosis in one anatomical location compared to another. A commonality in most pediatric brain tumor patients remains that what we accomplish as neurosurgeons in our attempts to remove a child’s tumor is presently the most important factor in that child’s prognosis. For most pediatric brain tumors, the difference between a grosstotal resection and a subtotal resection doubles the child’s chances of survival. As such, advances in neuroimaging and image-guided surgery are among the greatest adjuncts to surgery. The application of frameless stereotactic image guidance, functional neuroimaging, intraoperative MR imaging, early postoperative MR imaging, and second-look surgery has allowed the neurosurgeon to be more aggressive. This is evidenced by the fact that the number of children enrolled in national clinical trials with no or minimal residual tumor on their MR imaging has nearly doubled over the last 2 decades. Another major advance has been the routine banking of tumor tissue. This resource, coupled with more readily available molecular profiling techniques, has allowed us to understand that two ependymomas that look the same histologically are not the same tumor in the spinal cord as in the posterior fossa or the supratentorial space.8 The understanding of the molecular events giving rise to these tumors has allowed us to develop animal and in vitro models that are both histologically and molecularly the same as those in the human condition.4 The same holds true for small round blue-cell tumors. The recognition that molecular alterations in the INI-1 gene are associated with atypical teratoid rhabdoid tumors was a seminal occurrence.7 We now recognize that what we once called a medulloblastoma is one of several tumor subtypes.1 The Wnt-positive tumors, recognized by nuclear staining for b-catenin, are more likely to arise in younger children, are more likely to be located within the fourth ventricle, and in our last treatment trial were associated with 100% progression-free survival.2 Efforts are now underway to reduce the toxic side effects of treatment in this subgroup of children. Medulloblastomas associated with Sonic hedgehog (Shh) signaling abnormalities arise from a different cancer stem cell, are more likely to be located in the cerebellar hemisphere, and are more likely to be noted in infants and older children.3 With the development of GDC-0449, a specific Shh inhibitor, we now have targeted therapy for children with those medulloblastoma subtypes. For children with c-myc amplification or with large-cell anaplastic medulloblastomas, the prognosis remains dismal and we must develop novel targeted therapies for those children.5 In low-grade astrocytomas, similar progress has been made. The identification of BRAF abnormalities in pilocytic astrocytomas, the recognition that subependymal giant cell tumors respond quite nicely to mTOR inhibitors, and that antiangiogenesis agents such as bevacizumab offer specific molecular targets for treatment in these tumors, is further evidence that the understanding of the molecular pathways involved in tumor formation is our future. In this paper6 by Dr. Pollack, a pediatric neurosurgeon who has dedicated his life to better understanding childhood brain tumors, a very clear and readable overview of the progress made in the management of a variety of childhood brain tumors has been provided. A number of truths can be gleaned from the article. First, given the molecular studies that are available for these tumors, it behooves us at surgery not only to provide tissue for histology but also to bank fresh tumor tissue for future reference in each case. Second, children and their families should be offered enrollment in prospective clinical 133 134

Calcified multilocular thymic cyst associated with thymoma: a case report

IntroductionThere are few case reports of thymoma with a thymic cyst. Such an association renders it difficult for any pathologist to differentiate from other neoplasms, such as a cystic thymoma.Case presentationA 50-year-old Berber woman from Morocco was admitted with a chronic cough of more than 10 years duration. Her medical history and physical examination were normal. Anterior chest radiography demonstrated a calcified opacity in her right anterior mediastinum. A chest-computed tomogram revealed a round cystic tumor, with significant calcification in her right anterior mediastinum. A surgical exploration was performed. The tumor seemed to be a well-encapsulated and totally calcified lesion, arising from the right lobe of her thymus. It was removed by partial resection of her thymus. Through histology, the calcified tumor exhibited some areas of multilocular fibrous-wall cysts. These cysts were partially lined by small cuboidal cells with severe chronic inflammation and an AB thymoma that arose from the wall of the cyst.ConclusionGreater attention should be given to multilocular thymic cysts, to exclude the possibility of neoplasm, especially when the cyst wall is thickened.

Primitive Neuroectodermal Tumor of the Orbit in Adults: A Case Series

To present the clinical, radiologic, and histopathologic features of orbital primitive neuroectodermal tumor (PNET) in 5 adult patients. Retrospective case series of 5 adult patients with orbital PNET. Orbitotomy was performed in all cases. The authors report clinical findings, radiologic features, histopathology, immunohistochemical analysis, management, and outcomes for 5 patients with orbital PNET. Five adult patients presented with progressive unilateral proptosis and visual impairment. Common radiographic findings included a heterogeneous mass without associated destructive features, located in the superior and/or lateral orbit. Four cases demonstrated strong immunohistochemical staining for CD99 in a membranous pattern. One case required chromosomal analysis with fluorescence in situ hybridization to confirm the diagnosis. All patients received chemotherapy and/or orbital radiation with resolution of proptosis but no improvement of vision. One patient died of disease. To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.

Surgical Management of Lipoma in a Dog

Lipomas are benign fatty tumours composed of and each cell contain fat globule and nucleus pushed to mature fat cells. These neoplasms are commonly one side were observed. No mitotic figures were encountered in dogs, occasionally identified in cats observed. These findings confirmed as a case of and horses, and rarely observed in other domestic lipoma. species. (Aiello 1998). This paper reports about a case Discussion of lipoma in a dog. Lipomas are usually found in older and over Case history and Clinical Examination weight dogs and the incidence of neoplasms increase A Pomeranian dog aged around 6 years was with age (Moulton 1990). They may occur anywhere brought to clinic with the history of soft swelling on in the body, but commonly in the subcutis of the chest, ventral aspect of the lower part of the cervical region. abdomen, legs, and axillae. They may appear in the Initially, it was small in size and then subsequently orbital region, (Williams and Haggett 2006) perineal increased to the large lemon size with in a period of 3 region, (Besalti et al.2004), thigh region (Thomson et months. Clinical examination revealed a soft and al., 1999), and in the body cavity (Mayhew & round tumour on lower cervical region. On palpation, Brochman, 2002). Albers &Theilen (1995) suggested it was soft and painless. The clinical parameters like an alternate method of treatment by injecting 30% temperature, pulse rate and respiration rate were with calcicum chloride directly in to the subcutaneous in physiological limits. tumours. The dog made uneventful recovery and no Surgical treatment

Isolated primary schwannoma arising on the colon: report of two cases and review of the literature

Primary schwannoma of the large intestine is an extremely rare neoplasm. Here, we report two cases of colonic schwannoma confirmed pathologically after laparoscopic resection. A 52-year-old female and a 59-year-old female were referred by their general practitioners to our coloproctologic clinic for further evaluation and management of colonic submucosal masses. Colonoscopies performed in our institution revealed round submucosal tumors with a smooth and intact mucosa in the mid-ascending and descending colon, respectively. Computed tomography (CT) scans showed an enhancing soft tissue mass measuring 2 × 2 cm in the right colon and well-defined soft tissue nodule measuring 1.5 × 1.7 cm in the proximal descending colon, respectively. We performed laparoscopic right hemicolectomy and segmental left colectomy under the preoperative impression of gastrointestinal stromal tumors. Two cases were both diagnosed to be benign schwannoma of the colon after immunohistochemical stains (S-100 (+), smooth muscle actin (-), CD117 (-), and CD34 (-)).

Post-traumatic diaphragmatic herniation of the liver, examined by positron emission tomography: case report

We present a case of post-traumatic diaphragmatic herniation of the liver, which mimicked an intrathoracic tumor. After an automobile accident, the patient underwent thoracotomies for hemothorax and lung cancer in the right chest. Seven months later, computed tomography (CT) demonstrated a round tumor in the thorax adjacent to the right diaphragm with a higher density than the liver parenchyma. An intrathoracic tumor including a primary or metastatic lung cancer was suspected. However, positron emission tomography (PET) showed that the uptake of fluorine-18-fluorodeoxyglucose (FDG) was identical to that in the liver, and the tumor appeared to be contiguous with the liver. Thus, we suspected liver herniation. Core needle biopsy revealed liver cells without neoplastic tissue. Upon surgical exploration, herniation of the liver was found and repaired. PET was helpful in providing morphological and functional information leading to accurate diagnosis of liver herniation in this unusual case.

Pancreatic metastasis in a case of small cell lung carcinoma: Diagnostic role of fine-needle aspiration cytology and immunocytochemistry

Small cell lung carcinoma represents a group of highly malignant tumors giving rise to early and widespread metastasis at the time of diagnosis. However, the pancreas is a relatively infrequent site of metastasis by this neoplasm, and there are only occasional reports on its fine needle aspiration (FNA) cytology diagnosis. A 66-year-old man presented with extensive mediastinal lymphadenopathy and a mass in the pancreatic tail. Ultrasound-guided FNA smears from the pancreatic mass contained small, round tumor cells with extensive nuclear molding. The cytodiagnosis was metastatic small cell carcinoma. Immunocytochemical staining showed that a variable number of neoplastic cell were positive for cytokeratin, chromogranin A, neurone-specific enolase and synaptophysin but negative for leukocyte common antigen. The trans-bronchial needle aspiration was non-diagnostic, but biopsy was suspicious of a small cell carcinoma. This case represents a rare metastatic lesion in the pancreas from small cell lung carcinoma, diagnosed by FNA cytology.

Epidermoid Cyst of Round Ligament: Case Report and Review of Literature

Round ligament tumors are rare, and when found are typically leiomyomas. Herein is described a case of an epidermoid cyst of the round ligament, previously reported only once in the literature. Epidermoid cysts are similar to dermoid tumors but have only an epidermis-lined structure filled with keratin. Management included laparoscopic removal of the tumor. The patient’s recovery was uneventful.

Squash cytomorphology of central neurocytoma: A study of five cases

Intraoperative consultation is an important component in the surgical management of brain tumors. This study was undertaken to describe the cytomorphological features of central neurocytoma (CN) in squash smears. Squash smear of five CNs were reviewed. One to two millimeters of the biopsy material was crushed between two glass slides to make a thin film that was fixed in 95% alcohol and stained with haematoxylin and eosin (H&E). Cytological diagnoses were correlated with clinical and radiological data and finally with histopathological findings in the tumor. We had 5 cases (4 males, 1 female; age, 19-61 years; mean, 32 years). All tumors were located in the lateral ventricle. The smears were highly cellular displaying monomoprhic round tumor cells, round to oval nuclei with evenly distributed finely granular chromatin and ill-defined cytoplasmic borders within fibrillar matrix. Focal rosette formation is also noted. A capillary network was identified in between tumor cells in all the cases. One case had calcification with numerous psamomma bodies identified in the squash smear. The differential diagnosis is discussed. On squash smears, CN can be diagnosed when cytomorphological features are correlated with clinical and radiological finding.

Sclerosing epithelioid fibrosarcoma of the chest wall

ABSTRACTSclerosing epithelioid fibrosarcoma is an unusual variant of fibrosarcoma composed of epithelioid cells arranged in strands and nests in the background of a highly sclerotic collagenous stroma. Despite the relatively bland appearance and low mitotic activity, the tumor is capable of local recurrence and distant metastasis. We report a case of sclerosing epithelioid fibrosarcoma arising from the chest wall in a 26‐year‐old man. Microscopically, the tumor showed proliferation of round, oval, or angulated tumor cells in the sclerotic collagenous stroma. The tumor cells were small to medium in size, had pale eosinophilic cytoplasm and were arranged in cords, strands, and clusters. Immunohistochemically, the tumor cells were diffusely positive for vimentin, but negative for pan‐cytokeratin, S100 protein, human melanoma black 45, CD34, smooth muscle actin, and desmin. The tumor cells showed paranuclear dot‐like staining for beta‐catenin on immunostaining, but no nuclear staining was observed. The patient is alive 13 months after surgery.

Cytological findings of intrathyroidal epithelial thymoma/carcinoma showing thymus‐like differentiation: A study of eight cases

The purpose of this article is to describe the cytologic findings of intrathyroidal epithelial thymoma/carcinoma showing thymus-like differentiation (ITET/CASTLE) in detail and discuss its differential diagnoses. We examined cytologic specimens taken from eight ITET/CASTLE cases, who underwent fine needle aspiration. Cytologic features of ITET/CASTLE include (1) hypercellularity, (2) large cell clusters without papillary or follicular pattern, (3) round or spindle tumor cells with distinct nucleoli and cell border, (4) few keratinized cells and intracytoplasmic lumina (ICL), and (5) lymphocytic background. The differential diagnoses included poorly differentiated carcinoma, metastatic lymphoepithelioma, squamous cell carcinoma, and mucoepidermoid carcinoma. The presence of individual keratinizing cells and ICL and the location of the tumor may be helpful in indicating ITET/CASTLE.

Antenatal ultrasonographic features of fetal capillary hemangioma in the posterior fossa

A capillary hemangioma with hydrocephalus in the posterior fossa of a fetus was detected on ultrasonography at 38 weeks and 4 days of gestation. A well-defined, round tumor with a mixed pattern occupied the posterior fossa, and the normal cerebellum was significantly compressed by this tumor. No other anomaly was detected. Delivery was induced because of rapidly progressive hydrocephalus, and an otherwise healthy female infant weighing 2800 g was delivered vaginally at 39 weeks and 4 days of gestation. Histologic examination of the lesion through biopsy demonstrated capillary hemangioma. The tumor spontaneously decreased in size, and disappeared six months later. The child is now 2 years of age, and is developing normally.

Cecal Schwannoma: A Rare Case of Gastrointestinal Bleeding in a Young Woman

Purpose: Case Report: We present a case of a 35 year old woman who presented to our emergency department with epigastric pain and melena for two weeks. Abdominal pain was described as sharp in character, severe in intensity and worse after eating food. Patient also reported nausea, vomiting and some red blood in the stool for two days. She denied using non-steroidal anti inflammatory drugs. Medical history included gastroesophageal reflux disease, herniated disk, migraine headache and depression. Physical examination was unremarkable except for epigastric tenderness. Laboratory data showed hemoglobin of 9.2 g/d with iron deficiency. An upper gastrointestinal endoscopy performed did not reveal any pathology to explain the etiology of melena. A colonoscopy performed revealed a non-obstructing superficially ulcerated mass in the cecum. Biopsies of this mass only revealed an extensive focally necrotic ulceration of the colonic mucosa without any evidence of malignant cells. A computerized tomography scan of the abdomen identified a 4×4×3.5cm mass in the cecum. Surgical consultation was obtained. After discussion with the patient and her family a right hemicolectomy was performed. Operative findings revealed a large cecal mass, mesenteric lymphadenopathy and normal liver without evidence of any peritoneal metastasis. Pathology showed a round tumor comprising of cellular spindle cells in the cecum with prominent lymphoid cuffing. No mitosis or tumor necrosis was seen. Tumor cells were positive for S-100 protein on immuno-histochemical staining. A pathological diagnosis of Schwannoma of the cecum was made. The pathologist also reported an unremarkable appendix, margins of resection free of the tumor and twenty reactive lymph nodes negative for tumor. Post surgical recovery was uneventful. Subsequent follow-up in the clinic was without any new symptoms or complications. Discussion: Schwannomas of the colon and rectum are very rare tumors that are derived from the Schwann cells of the neural sheath. Review of literature reveals only a small number of reported cases. These tumors can present as polypoid intraluminal lesions. They may cause rectal bleeding, colonic obstruction, defecation disorders or abdominal pain. A definitive pathological diagnosis is difficult to make preoperatively. Radical excision with margins free of tumor remains the treatment of choice. Our case illustrates an example of a very rare tumor presenting with melena in a young patient requiring surgery.

A nodular lesion of the toe

Conflict of interest: none declared. A 40‐year‐old Afro‐Caribbean man presented with a 5‐year history of a growth on the left second toe, displacing the nail plate. The lesion had gradually been increasing in size, but in the past year had grown more rapidly. It was painful if the patient was wearing tight footwear. There was no preceding history of trauma. On examination, there was a firm round tumour, 20 mm in size, displacing and distorting the whole nail plate was seen (Fig. 1a). It was tender on palpation. No underlying exostosis was seen on radiographs. A biopsy was taken from the lesion. ... Histological examination of the biopsy revealed a dermal and subcutaneous tumour consisting of bland stellate and elongated spindle‐shaped cells. These were arranged in a vaguely storiform and fascicular growth pattern in a myxoid stroma, with some increase in mast cells. There were scattered small blood vessels. No mitotic figures were seen (Fig. 1b).

Tumor desmoplásico de pequenas células redondas abdominal da infância: relato de caso

O tumor desmoplásico de pequenas células redondas (TDPCR) é uma neoplasia rara e altamente agressiva, que afeta predominantemente jovens do sexo masculino. Relata-se um caso de TDPCR em um paciente do sexo masculino, de 11 anos, com acometimento intra-abdominal marcado por volumosa massa retroperitoneal em hipocôndrio esquerdo. O estudo histológico da massa revelou presença de blocos de pequenas células tumorais redondas e azuis, envoltas por estroma desmoplásico; a análise imuno-histoquímica evidenciou positividade para desmina, WT-1 e citoceratinas. Após o diagnóstico, o paciente foi submetido a tratamento quimiorradioterápico, tendo evoluído a óbito durante o 24º mês de acompanhamento.

Primary pulmonary small leiomyosarcoma arising from bronchial smooth muscles

A 74-year-old woman was admitted in an hospital because of abnormal lung shadow in chest X–P. Imaging modalities including CT, MRI, and PET demonstrated a small (12 mm in diameter) mass in the periphery of the right upper lobe. Although biopsy and cytology were negative, wedge resection was performed. Grossly, the lesion was a round white tumor measuring 10 × 10 × 9 mm located beneath the pleura. Microscopically, the tumor consisted of proliferation of atypical spindle cells with hyperchromatic nuclei. The tumor cells were continuous with bronchial smooth muscle. Mitotic figures were recognized in 20 per 10 high power fields. There were foci of necrosis and deposition of dusts and hemosiderin. Residual bronchi were present within the tumor. Immunohistochemically, the tumor cells were positive for vimentin, α-smooth muscle actin, caldesmon, and Ki-67 (labeling = 50%), but negative for cytokeratins (AE1/3, CAM5.2, KL-1, and polyclonal wide), cytokeratin 5/6, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, melanosome, TTF-1, desmin, CD34, calreninin, D2-40 (podoplanin), S100 protein, p53 protein, myoglobin, KIT, and platelet-derived growth factor receptor-α (PDGFRA). The author diagnosed the lesion as leiomyosarcoma. After the pathological diagnosis, CT, MRI and PET of the whole body were performed, but did not reveal any tumors including the uterus. The patient was followed up, and three years later a recurrent lesion (20 mm in diameter) was identified in the right upper lobe near the previous tumor. Biopsy showed leiomyosarcoma with histology and immunohistochemical findings similar to the previous tumor. Further operation was impossible because of her medical conditions. She was treated by chemotherapy and followed up. She was now alive with the small pulmonary tumor, which shows no rapid growth.

Primitive Neuroectodermal Tumor of the Liver: A Case Report

Ewing sarcoma/primitive neuroectodermal tumor is a rare tumor of soft tissues of thoraco-pulmonary regions, pelvis and lower extremities. Involvement of visceral organs by primitive neuroectodermal tumor is even rarer, with the kidney being the most commonly involved organ. Involvement of the liver has been reported in the form of metastasis from other primary sources presenting as liver abscess. We report a 20-year-old lady presenting with massive hepatomegaly, with computed tomography scan evidence of diffuse hepatomegaly and a normal porta and intrahepatic biliary radicles. She subsequently underwent ultrasonography-guided true-cut needle biopsy of the liver. Histopathology of the liver revealed nests of small round blue tumor cells in the background of hepatocytes infiltrating the liver, which expressed Mic-2 and Fli-1, and were negative for cytokeratin, desmin, hepatocyte-specific antigen (OCHIE5), synaptophysin, chromogranin A and CD-20. Immunohistochemistry revealed CD-99-positive. Extensive search regarding any possible different site of involvement by the tumor was negative. The patient responded to a combination therapy of vincristine, adriamycin and cyclophosphamide alternating with ifosfamide and etoposide 3 weekly over 43 weeks and has been doing well even after 1 year of diagnosis. The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy.

A 2-year-old boy with a stage III yolk sac tumor occurring in an intra-abdominal retained testis

A major complication of retained testes is an occurrence of malignancy later in life. We, herein, report the case of a 2-year-old boy who presented with a huge yolk sac tumor with retroperitoneal lymph nodes metastasis that originated in a left intra-abdominal undescended testis. Computed tomography and magnetic resonance imaging showed a huge round tumor connecting to the left retroperitoneal lymph nodes with metastasis extending from the left pelvic region to the left renal hilum. The serum α-fetoprotein level was 36,528 ng/mL. The right abdominal tumor appeared to be a giant testis that had strangulated at the neck of the cord. The tumor had ruptured at the side of the left pelvic lymph node metastasis, and a yolk sac tumor was diagnosed from a histologic analysis of the resected specimens. Postoperative PEB chemotherapy was effective, and a complete surgical resection of the tumor was performed 3 months after the initial laparotomy. The pathologic findings showed fibrous tissue without any tumor cells. The patient has been doing well for 18 months after the radical operation. This case might be a coincidental association of a yolk sac tumor occurring in an undescended testis, which thus caused a delay in making an accurate diagnosis.