Enhancing Soft Tissue Mass Research Articles

Schwannoma of Ansa-Cervicalis: A Rare Location for Schwannoma.

Schwannomas are rare neoplasms that originate from the nervous system. We describe a case of schwannoma of the ansa-cervicalis in a patient where preoperative imaging is suggestive of glomus caroticum tumor. A 44-year-old man presented to the surgical oncology department with a recurrent anterior triangle neck swelling. Magnatic resonance ımaging (MRI) demonstrated right enhancing soft tissue mass at the carotid bifurcation consistent with glomus tumor. Intraoperatively, the lesion was found abuting the bifurcatuion and arising from the right ansa-cervicalis. Histopathology evaluation revealed schwannoma. Schwannoma of the ansa-cervicalis is extremely rare. Very few cases have been reported in the literature. Making the preoperative diagnosis is difficult. Preoperative imaging is necessary to rule out other causes of neck masses such as thyroid lesions, lymphadenopathy, and carotid body tumor. Verocay bodies were noted on histology. Schwannoma of cervical region can have origin from any nerve. Any Neurologic post-operative deficit depends on the nerve of origin and the precision of the surgery performed.

Breast Carcinoma with Isolated Orbital Metastasis –A Rare Clinical Entity

Breast cancer with isolated orbital metastasis is a rare clinical entity.We hereby present a rare case report of 35 year old premenopausal lady with known case of carcinoma breast right side (operated for right modified radical mastectomy in year 2017 with final histopathology of pT2N2M0, hormone receptor-positive and Her2-negative disease). She completed adjuvant 8 cycles of standard dose chemotherapy with 4 cycles of Cyclophosphamide, Adriamycin followed by 4 cycles of Paclitaxel followed by local Radiation therapy. She was initiated Tab. Tamoxifen 20 mg once daily and was continued same for 4 years when in July 2021, she complained of sudden decreased visual acuity and diplopia of right eye lasting from last 1 month. Ophthalmological examination confirmed the findings with decreased visual acuity of 6/36 of right eye while visual acuity of left eye was normal. CT scan of orbits revealed 28 × 18mm heterogeneous enhancing soft tissue mass in right orbital compartment,extrabulbar, occupying postero-superior quadrant inseparable from lateral rectus muscle with optic nerve effacement. PET-CT scan was performed which revealed no metastasis elsewhere in the body. She was offered surgical oophorectomy followed by switch to another hormonal therapy of aromatase inhibitors. She showed complete response to treatment and is now disease from last 2 years.

Retinoblastoma Extending up to the Optic Chiasma and Presenting as a Suprasellar Mass

Retinoblastoma is the most common ocular malignancy of childhood. It is present in childhood with leukocoria and strabismus. Most patients are diagnosed under 3 years of age. Funduscopic examination can reveal an intraocular mass, but imaging is essential for complete evaluation of the lesion. Although ultrasound is a non-invasive and relatively inexpensive screening tool, cross-sectional imaging is required to assess the involvement of optic nerve and intracranial spread. We report a case of retinoblastoma in a 5 year old male child who presented with headache diminution of vision in both eyes. Contrast enhanced magnetic resonance imaging (MRI) revealed an enhancing soft tissue mass in the right eye with involvement of optic nerve and optic chiasma and contiguous with a large suprasellar mass causing hydrocephalus. Non-contrast computed tomography (CT) showed extensive calcifications in the mass lesion.

UNUSUAL DIAGNOSIS OF A MEDIASTINAL MASS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Paragangliomas are rare neoplasms with an annual incidence of 0.8 per 100,000-person years [1]. Mediastinal paragangliomas represent only 2% of paragangliomas [2]. However, with increased availability of imaging, the number of these neoplasms found incidentally in asymptomatic individuals is increasing. This case report presents an asymptomatic paraganglioma in the mediastinum. CASE PRESENTATION: 51-year-old white female with a history of hypertension and anxiety who presented with worsening shortness of breath, weakness, fever, chills and nonproductive cough. The patient was admitted because she failed outpatient treatment with amoxicillin/clavulanate. CT chest revealed basilar consolidation and a bulky centrally necrotic subcarinal nodal mass. She was discharged 5 days later after one dose of dexamethasone as well as a course of IV azithromycin and ceftriaxone. Repeat CT chest 6 weeks later showed a persistent enhancing soft tissue mass in the mediastinum measuring 4.8 x 2.9 cm. Pulmonology was consulted and performed an endoscopic bronchial ultrasound biopsy with fine-needle aspiration that revealed mediastinal lymphadenopathy as well as a partial narrowing from mass effect in the left main bronchus, left lower lobe and the lingula. Histologic sections and immunohistochemistry confirmed the diagnosis of paraganglioma. The patient was sent to thoracic surgery to consider resection and is currently awaiting workup with endocrinology. DISCUSSION: Mediastinal paraganglioma is a rare tumor and often because of their location biopsy to confirm diagnosis is often delayed. Half of patient's are asymptomatic [3] and may only be diagnosed incidentally or once mass effect produces clinical symptoms to initiate workup. Clinical symptoms in symptomatic patients are often from catecholamine hypersecretion causing palpitations, sweating, headaches and most commonly hypertension. Non-catecholamine symptoms may include hoarseness, shortness of breath or dysphagia depending on the location of the tumor. Surgical tumor removal remains the cornerstone of treatment. However, resection may not be feasible due to these tumors being highly vascular and neighboring critical vascular structures. CONCLUSIONS: Paraganglioma is a rare tumor in the mediastinum that is usually asymptomatic and found incidentally. Surgical resection can be curative. REFERENCE #1: Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc. 1983 Dec;58(12):802-4. PMID: 6645626. REFERENCE #2: Ghayee HK, Havekes B, Corssmit EP, et al. Mediastinal paragangliomas: association with mutations in the succinate dehydrogenase genes and aggressive behavior. Endocr Relat Cancer 2009; 16:291. REFERENCE #3: Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc. 1981 Jun;56(6):354-60. PMID: 6453259. DISCLOSURES: No relevant relationships by Haytham Adada, source=Web Response No relevant relationships by Mahmoud Amarna, source=Web Response No relevant relationships by Camelia Chirculescu, source=Web Response No relevant relationships by Trevor Elswick, source=Web Response

Smooth Muscle Tumor of Uncertain Malignant Potential Arising from Renal Vein

Smooth muscle tumors (SMTs) typically fall under either benign leiomyoma or malignant leiomyosarcomas based on histologic characteristics. SMTs that cannot be diagnosed as benign or malignant are then labeled as smooth muscle tumors of uncertain malignant potential (STUMP). A 31-year-old male presented with the right abdominal pain for 6 months. Imaging showed an enhancing soft-tissue mass arising from the right renal vein. A pre-operative biopsy confirmed STUMP of renal vein, and the patient underwent surgical resection of the mass with partial nephrectomy. Imaging and pathologic findings play a crucial role in an accurate pre-procedural diagnosis of unusual SMTs.

S3568 Metastatic Jejunal Carcinoid Masquerading as a Pelvic Mass

INTRODUCTION: Neuroendocrine tumors (NETs), also known as carcinoid tumors, are epithelial neoplasms that arise from enterochromaffin cells and are classified according to their histologic features and confirmed by immunohistochemical staining. NETs most commonly occur in the gastrointestinal tract, pancreas, appendix, rectum and the lungs but they can develop anywhere in the body. Due to their common embryonic origin, neuroendocrine tumors of the jejunum, ileum and appendix are collectively called midgut carcinoid tumors. NETs are now the most common small bowel tumor. Small bowel NETs are difficult to diagnose as they follow an insidious course; early on in the disease, symptoms are vague and nonspecific. Late diagnosis when regional or distant metastasis have already occurred is common. Forty percent of symptomatic patients without carcinoid syndrome present with vague abdominal pain. CASE DESCRIPTION/METHODS: We present a case of a 58 yo female with complaints of abdominal pain. The patient had six months of alternating fevers, chills and fatigue. She was placed on antibiotics but her fevers returned upon completion. Three months thereafter, the patient developed left lower quadrant abdominal pain that was sharp in nature and radiated to her groin. A CT scan of the abdomen and pelvis demonstrated an enhancing soft tissue mass with internal cystic and necrotic changes located in the midline upper pelvis, measuring 2.8 by 2 cm and two adjacent satellite nodules located superior to the mass. The location of this mass made it un-amenable to a percutaneous biopsy. The patient underwent an Endoscopic Ultrasound Guided Fine Needle Aspiration (EUS-FNA) with cytology demonstrating low grade neuroendocrine carcinoma with the malignant cells being positive for chromogranin A and synaptophysin. Endoscopic evaluation was negative. A capsule endoscopy revealed two small areas in the jejunum that were concerning for small bowel tumors. A subsequent octreoscan was positive, showing an abnormal activity in the mid-abdomen. The patient subsequently underwent surgical excision. The surgical pathology confirmed the mesenteric mass finding as metastatic low grade neuroendocrine tumor and identified two distinct small bowel low grade carcinoid tumors. DISCUSSION: Carcinoid tumors can vary in their presentation and delay the time of diagnosis. Early consideration for NETs in the differential for patients with vague undiagnosed abdominal symptoms could lead to more prompt diagnosis and treatment prior to metastatic spread.Figure 1.: Tumor Positive for Chromogranin A.Figure 2.: Tumor Positive for Synaptophysin.

MON-255 Oral Contraceptive Pills Mask an Aggressive Crooke’s Cell Pituitary Adenoma

BackgroundCrooke’s cell tumors are rare and aggressive forms of pituitary adenomas. This variant of Cushing’s disease requires prompt diagnosis to avoid life-threatening complications. We report a unique case of Crooke’s cell tumor with longstanding history of irregular menstrual cycles, undiagnosed and later presented as acute unilateral ptosis and diplopia due to aggressive tumor invasion.Clinical Case23-year-old female presented to the ER with facial swelling, left eye droop and diplopia for 3 days. She had a past medical history of oligomenorrhea and hirsutism which was normalized by oral contraceptive pills (OCP)- a combination of ethinyl estradiol and drospirenone for the last 3 years. Years prior, workup of her oligomenorrhea showed normal androgenic profile with normal DHEA-S, testosterone and 17-OH progesterone. Current exam was also significant for elevated blood pressure 200/110mmHg, BMI 37, pigmented abdominal striae and terminal hair over her chin. Labs remarkable for hypokalemia K+ 2.7 mmol/L (3.5-5.3), elevated AM cortisol 51 mcg/dL (4-20), low TSH 0.152 mcUnit/mL (0.4-5.0), low IGF-1 170 ng/mL (222-566) and FSH 1.4 mUnit/mL (1.0-9.0), with normal prolactin 24.3 ng/mL (<0.5-25) and free T4 0.87ng/dL (0.8-1.8). MRI brain showed 2.8cm homogenous enhancing soft tissue mass involving the central skull base, sphenoid sinus, sella, suprasellar cistern, and parasellar regions; displacing the optic chiasm, and invading the cavernous sinuses bilaterally and orbital apices. Post trans-sphenoidal surgery (TSS) of the pituitary mass, her left eye ptosis and diplopia resolved. Post-op MRI showed subtotal resection of the extra-axial enhancing abnormality at the central skull base with extension to multiple other anatomic spaces. Pathology read consistent with aggressive Crooke’s cell adenoma, showing invasive biologic behavior without an elevated proliferation index with positive ACTH immunohistochemistry supportive of corticotroph cell adenoma. Post-op ACTH level 73 pg/mL (6-50) and cortisol 12.5 mcg/dL (4-20), while on dexamethasone. Repeat TSS was performed for residual adenoma. Cortisol remains elevated at 15.7mcg/dL despite high dose dexamethasone taper by the neurosurgery team for post-op development of right eye ptosis. She is currently awaiting proton beam radiation therapy.ConclusionCrooke’s cell tumors are an aggressive form of pituitary adenoma for which early diagnosis is crucial for its prognosis. Our case highlights the importance of maintaining a wide differential in evaluating young women with menstrual irregularities and to include screening for Cushing’s syndrome. Empiric treatment with OCPs can mask symptoms in the earlier course of Cushing’s disease as in our patient, causing recognition only after presentation with significant tumor growth. Earlier detection could have prevented adenoma invasion and potential neurological sequelae.

Merkel Cell Carcinoma: A Case-Report of Rare Presentation of Merkel Cell Carcinoma, Non-Indolent Proliferative Type, Polyomavirus-Negative: Hypothesis of MolecularPathogenesis

Introduction: Merkel cell carcinoma (MCC) is a rare cutaneous indolent neuroendocrine cancer, arising from the Merkel cells of the stratum basalis, of the epidermis. This type of tumor commonly arises in sun-exposed areas, such as head, neck, and lower extremities. Here, we describe a rare presentation of non-indolent proliferative type Merkel cell carcinoma. Case Report: This is a case-report of a 70-year-old Caucasian man with no pertinent past medical history, presenting as a large painless violaceous necrotic irregular bordered rapidly growing lesion, reaching to 8x10x15 cm3, within one-year period, on the lower back region with surface ulceration. Pelvic MRI showed a multilobulated enhancing soft tissue mass measuring 8.7x10.4x15.1 cm3 at the left gluteal region with exophytic extension to the left paraspinal muscles. The patient was admitted, tangential surgical excision and debridement of left flank wound was performed with partial primary closure. Pathology showed features of Merkel cell, show diffuse dot-like positivity with CK20 and are negative for CK7, there is diffuse positivity with NSE, synaptophysin and CD56 with strong diffuse Ki-67 positivity noted in &gt;65% of tumor cells. CD99 shows diffuse small faint dot-like paranuclear positivity. Discussion: Merkel cell carcinoma (MCC) is a rare, aggressive tumor that generally arises in sun-exposed regions. After an initial course of slow growth, starting as a painless violaceous non-pruritic domed-shaped lesion, the tumor becomes more aggressive, rapidly growing, with metastasis with local lymph nodes and regional tissue invasion. Sixty percent of tumors can rapidly grow within a three-month period after initial diagnosis. MCC is also clonally associated with is polyomavirus. MCC Contributing molecular pathogenesis is imperative to determining the causation of rare non-indolent MCC tumors, and its association with prognosis and treatment. In polyomavirus negative patients, consideration for molecular pathogenesis as etiology is imperative.

1498 A Rare Case of an Atypical Mass Involving the Sigmoid Colon

INTRODUCTION: The etiology of colonic masses is broad including benign polyps, primary adenocarcinoma, metastases, and diverticular mass. Presentation may be similar; therefore, the differentiation generally involves biopsy for histological evaluation and appropriate diagnosis. CASE DESCRIPTION/METHODS: A 34-year-old female presented with hematochezia and constipation. Her past medical history was significant for tubo-ovarian ovarian abscesses (TOAs), endometriosis fibroids and schwannoma of the paraspinal area. She reported new onset red blood per rectum, worsening constipation, and pelvic discomfort. Family history was notable for esophageal and colorectal cancer. She subsequently underwent colonoscopy which demonstrated a 3-4 cm nodular, polypoid fungating mass in the distal sigmoid colon located at 20-25 cm from the anal verge (Figure 1). Biopsies were obtained and the site was tattooed. A subsequent MRI of the abdomen and pelvis showed a heterogeneous inflammatory soft tissue encasing the ovaries and tethering the rectosigmoid colon (Figure 2). Additionally, there was a enhancing soft tissue mass invading the inferior wall of the distal sigmoid colon with an intraluminal component. The MRI findings were felt to be consistent with invasive endometriosis. Histological examination of the sigmoid mass showed colonic mucosa with polypoid endometriosis without evidence of malignancy (Figure 3). Cells showed positive immunohistochemical staining consistent with endometriosis. Surgical resection was recommended. DISCUSSION: This case demonstrates the importance of having a high suspicion of endometriosis involving the bowel in reproductive age women presenting with abdominal pain, dysmenorrhea, dyspareunia, and dyschezia. Endometriosis is a condition involving the implantation of endometrial stroma and glands at extra-uterine sites. Endometriosis tends to involve bowel serosa. It rarely encompasses full thickness of the bowel wall. The latter represents invasive endometriosis. Many patients are incorrectly diagnosed given their presentation. This disease increases risk of gastrointestinal tumors, primarily adenocarcinoma. Therefore, prompt recognition and treatment with hormonal therapy or surgical excision is important for symptom relief, increase in fertility, and later complications such as bowel stenosis, hydronephrosis, and malignant transformation.

EPITHELIOID RHABDOMYOSARCOMA OF THE ORAL CAVITY, NOVEL LOCATION OF A RARE ENTITY: A CASE REPORT

<h3>Background</h3> Rhabdomyosarcoma, a malignant neoplasm, is characterized by skeletal muscle differentiation. Only 33 cases of the epithelioid variant have been reported, and just 8 of them were located in the head and neck region. This aggressive entity usually presents as a fast-growing, asymptomatic infiltrative mass and presents predominantly in adults. A 53-year-old male, who was edentulous and a former smoker, presented with enlargement of 2 months' duration in the right maxillary vestibule. A clinically palpable mass was noted under the mucosa. No adenopathy or cranial nerves deficit was observed on physical examination. The initial panoramic radiograph showed a corticated radiolucent entity located on the right maxilla, with extension into the right maxillary sinus. Multidetector computed tomography (MDCT) and positron emission tomography/computed tomography (PET/CT) were performed. CT revealed an enhancing soft tissue mass of the right alveolar ridge with extension to the maxillary sinus, with erosion of the anterior and lateral walls of the sinus and the adjacent alveolar ridge. PET/CT showed a right maxillary fluorodeoxyglucose (FDG) high uptake and right neck level IIA lymph nodes with mild uptake. An incisional biopsy was performed. The histologic appearance and immunohistochemical profile supported a diagnosis of high-grade epithelioid rhabdomyosarcoma. The treatment protocol was right maxillectomy and right neck dissection with coadjuvant radiation therapy. After 1.5 years, follow-up CT was performed and did not show any signs of recurrence or metastatic disease. <h3>Discussion/Conclusions</h3> Epithelioid rhabdomyosarcoma is a rare variant of rhabdomyosarcoma. To the best of our knowledge, none has been reported in the oral cavity region; thus, this is the first case of this aggressive variant described in this location. This entity may develop without any apparent symptoms or signs of a malignant entity, thus mimicking the appearance of a benign neoplasm or inflammatory process. A comprehensive multidisciplinary approach is advised to diagnose and treat this entity accordingly.

Unusual Case of Esophageal Adenocarcinoma With Forearm Soft Tissue Metastasis

The patient is a 66 year old male with a past medical history of diverticulitis and hypertension who presented with dysphagia and heartburn. Initial CT of the chest showed a thickened distal esophagus (Fig 1). EGD showed a large fungating mass that was partially obstructing and circumferential with bleeding in the lower third of the esophagus. This was stented. There was another medium sized ulcerated partially circumferential mass with no bleeding found in the cardia. Pathology of the cardiac mass revealed infiltrating adenocarcinoma histologic grade 2-3. Biopsy of the lower third of the esophagus showed infiltrating adenocarcinoma histologic grade 3. Follow up PET scan 2 months after diagnosis showed a focal hypermetabolic lytic lesion in the left femoral neck compatible with active tumor (Fig 2). Left femoral head arthroplasty for impending fracture was performed, with pathology revealing metastatic adenocarcinoma with esophageal primary. 2 months later, the patient complained of a soft tissue mass in the left forearm. MRI showed an enhancing soft tissue mass involving the flexor compartment of the proximal forearm and extending 13 cm longitudinally. Imaging appeared to be consistent with neoplasm with encasement of the ulnar artery and nerve, as well as displacement of the radial artery (Fig 3). Fine needle aspiration and core biopsy revealed adenocarcinoma with mucinous features and signet ring cells. Esophageal adenocarcinoma has been known to metastasize to bone, as was found in our patient who had femur head metastases. However, there are extremely few cases of metastases to the soft tissue. The pathophysiology of this is thought to be similar to skeletal muscle and cardiac muscle metastases - hematogenous spread. There have been other factors implicated in the phenomenon, such as pH, accumulation of metabolites, and local temperature of the soft tissue sites. However, as this is extremely rare, they are just hypotheses. Another interesting aspect of the case was the pathology from this specimen showed signet ring cells, which were not detected on any of the other biopsy results. Signet ring cells are only found in approximately 5% of esophageal adenocarcinomas, but portend a much more aggressive biologic behavior demonstrated by the rapid onset of symptoms and diagnosis. This is one of the first few cases in the literature to show metastatic esophageal adenocarcinoma to soft tissue.Figure: The left image is an axial view of the thoracic cavity showing circumferential thickening of the esophageal wall. The right image is a coronal view capturing the esophageal stent.Figure: This is a PET scan, in which there is increased focal uptake at the left femoral neck with an SUV max of 7.9.Figure: This image shows the 13cm x 7cm x 5cm enhancing soft tissue mass involving the flexor compartment of the proximal forearm, encasing the ulnar artery and nerve, and displacing the radial artery.

Primary Non-Hodgkin’s Orbital lymphoma of diffuse large B-cell type: a rare presentation

Primary Non-Hodgkin’s lymphoma of the orbit is a rare presentation and the diffuse large B-cell lymphoma (DLCL) type of histology is much less commoner than the mucosa associated lymphoid tissue (MALT) and follicular lymphoma. A 70 years female patient presented with palpable mass arising from right orbit and proptosis of the right eye. CT scan suggested homogenous enhancing soft tissue mass affecting right lacrimal region without any bony destruction. Biopsy confirmed it to be a case of Primary non-Hodgkin’s lymphoma of diffuse large B-cell type with strongly positive CD 20. She was given 6 cycles of chemotherapy with R-CHOP after surgery. The patient is now asymptomatic one year after the last cycle of the chemotherapy.Bangladesh Journal of Medical Science Vol.15(4) 2016 p.624-627

PNR-05PRIMITIVE MYXOID MESENCHYMAL TUMOUR OF INFANCY – A CASE OF EARLY CHEMOTHERAPY-RESPONSIVENESS AND REVIEW OF THE LITERATURE

PNR-05. PRIMITIVE MYXOID MESENCHYMAL TUMOUR OF INFANCY – A CASE OF EARLY CHEMOTHERAPYRESPONSIVENESS AND REVIEW OF THE LITERATURE Charlotte Burns1, Juliet Clayton2, Jessica Ng3, and Jordan Hansford1; The Children’s Cancer Centre, The Royal Children’s Hospital, Melbourne, Australia; The Department of Neurosurgery, The Royal Children’s Hospital, Melbourne, Australia; The Department of Anatomical Pathology, The Royal Children’s Hospital, Melbourne, Australia Primitive myxoid mesenchymal tumour of infancy (PMMTI) is a newly described mesenchymal tumour occurring within the first year and with a predilection for deep soft tissue around the spinal cord. Histologically characterised by proliferation of primitive immature mesenchymal cells and distinctive myxoid background, it is rare with less than twenty reported cases. Complete surgical resection provides optimal disease control, as tumours are locally aggressive and recur early. Rarely, metastases or transformation to higher-grade lesions has been observed. Systemic therapy has added little value to date. We presentaneonatewith PMMTI andanearly response tochemotherapy.A term neonate admitted at twenty-one days old with bronchiolitis was noted to have progressive lower limb weakness, urinary retention and reduced anal tone. MRI spine showed an enhancing soft tissue mass within the spinal canal extending fromL1-L5. Completemacroscopic surgical resectionwasperformed. Histologywas consistentwith PMMTI. Within threemonths, progressiveneurological deficits recurred. Repeat MRI revealed unresectable, extensive local recurrence with intraand extra-spinal components and biopsy confirmed identical histology. With limited therapeutic options available, chemotherapy was initiated (ifosfamide, carboplatin and etoposide, followed by vincristine, actinomycin, cyclophosphamide and doxorubicin). Therewasmarked clinical improvement after one cycle and 35% tumour volume reduction on MRI after two. Chemotherapy-responsiveness to this extent has not been documented to our knowledge. We present a case of PMMTI presenting typically, but with an early response to aggressive sarcoma-based chemotherapy. This treatment could be considered for unresectable PMMTI, an increasingly recognised entity, in the future. Neuro-Oncology 18:iii7–iii15, 2016. doi:10.1093/neuonc/now067.5 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Is cholesteatoma a precursor of verrucous carcinoma: A diagnostic dilemma

Verrucous carcinoma is an unusually well-differentiated variant of squamous cell carcinoma, characterized by local invasion and cytologically benign appearance. We report a case of 52-year-old male who presented with postauricular growth and cholesteatoma in the right ear. High-resolution computed tomography temporal bone showed an enhancing soft tissue mass lesion extending into the mastoid, external auditory canal, and middle ear. Growth was histopathologically confirmed to be verrucous carcinoma extending from the cholesteatoma in mastoid cavity. Review of literature confirms the rarity of cases of cholesteatoma which could progress and present as verrucous carcinoma in the postauricular region.

Paediatric Parameningeal Rhabdomyosarcoma: A Case Report Post-multimodal Treatment

Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood, representing 5% of all childhood cancers. We are reporting an interesting case of parameningeal rhabdomyosarcoma in a child who underwent multimodal treatment. A 7 year old boy presented to our clinic with a history of bad breath, nasal obstruction and recurrent epistaxis from the left nostril for 3 months. On examination he had mild left proptosis with normal eye vision and movements, reddish left nasal mass with a smooth surface. Paranasal CT scan showed slightly enhancing soft tissue mass 72×77 mm in the nasal cavity that deviated the nasal septum to the right, extending to the nasopharynx posteriorly and to the maxillary and ethmoidal sinuses with another mass 11×16 mm extension into the orbit. Biopsy was taken and histology showed embryonal rhabdomyosarcoma. An extensive tumour debulking was done folwed by chemotherapy (vincristine, dactinomycin and cyclophosphamide) and 50 grays of radiotherapy. A surveillance CT scan a month after treatment revealed over 90% tumour reduction. All patients with metastatic disease (group IV, stage 4) are considered high risk, except children and adolescents younger than 14 years with embryonal rhabdomyosarcoma. Advances into the multimodality management have dramatically improved survival in PM-RMS from approximately 25% to 75%.

Paediatric paramenigeal rhabdomyosarcoma: a case report – multimodal treatment

Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood, representing 5% of all childhood cancers. We are reporting an interesting case of parameningeal rhabdomyosarcoma in a child who underwent multimodal treatment. A 7-year-old boy presented to our clinic with a history of bad breath, nasal obstruction and recurrent epistaxis from the left nostril for 3 months. On examination he had mild left proptosis with normal eye vision and movements, reddish left nasal mass with a smooth surface. Paranasal CT scan showed a slightly enhancing soft tissue mass 72 × 77mm in the nasal cavity that deviated the nasal septum to the right, extending to the nasopharynx posteriorly and to the maxillary and ethmoidal sinuses with another mass 11 × 16mm extension into the orbit. Biopsy was taken and histology showed embryonal rhabdomyosarcoma. An extensive tumour debulking was done followed by chemotherapy (vincristine, dactinomycin and cyclophosphamide) and 50 grays of radiotherapy. A surveillance CT scan a month after treatment revealed over 90% tumour reduction. All patients with metastatic disease (group IV, stage 4) are considered high risk, except children and adolescents younger than 14 years with embryonal rhabdomyosarcoma. Advances into the multimodality management have dramatically improved survival in PM-RMS from approximately 25% to 75%.

A Rare Cause of Gastrointestinal Bleeding

62 Question: A 60-year-old woman presented to our emergency room with epigastric fullness and passage of tarry stool for 1 week. She also had progressive shortness of breath, dizziness, and generalized weakness. On physical examination, slight tachycardia (heart rate, 104 bpm) with anemic conjunctiva was noted. Blood tests showed low hemoglobin (6.9 g/dL). Esophagogastroduodenoscopy revealed a polypoid tumor with superficial ulcerations and hematin coatings, suggesting recent bleeding, near superior duodenal angle of duodenum (Figure A). A barium study showed a filling defect over mesenteric side of second portion of duodenum, resulting in luminal narrowing (Figure B, black arrow). Biopsy at the overlying mucosa showed inflammatory changes without neoplastic cells. Abdominal computed tomography disclosed an enhancing soft tissue mass in the second portion of the duodenum (Figure C, D, white arrow). Tumor markers (carcinoembryonic antigen and carbohydrate antigen 19-9) were within normal limits. The patient underwent pancreaticoduodenotomy with limited antrectomy to remove the tumor. What is your diagnosis of this duodenal tumor? Look on page 261 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

Desmoid Tumor (Fibromatosis) of the Breast after Augmentation with Saline Implants

Sir:FigureIn June of 2010, a 38-year-old Vietnamese woman returned to our plastic surgery clinic with complaints of a lump in the upper outer quadrant of her left breast. She had previously undergone bilateral augmentation mammaplasty with submuscular saline implants in 2006. On physical examination, she had a palpable mass in the 2-o'clock position near the anterior axillary line. However, it was not visually perceptible (Fig. 1). Magnetic resonance imaging revealed an enhancing soft-tissue mass (Fig. 2). Open capsulotomy was performed, and a 3.5 × 3 × 1.3-cm tumor was excised. The previous saline implants were intact, and they were exchanged for silicone implants bilaterally.Fig. 1: Bilateral saline implants and unresected left breast desmoid tumor.Fig. 2: T2-weighted magnetic resonance imaging scan with left breast desmoid tumor marked with a white arrow.Sectioning revealed an encapsulated rubbery tan mass. Microscopically, there were spindle cells against a background of scant collagen and occasional mitosis, consistent with a desmoid tumor (fibromatosis). Immunohistochemical staining for β-catenin was strongly positive, solidifying the diagnosis. Surveillance computed tomographic scans obtained at 6-month intervals for 12 months have remained unchanged with observation. There are fewer than 250 cases of breast desmoids in the literature. They are believed to arise from musculoaponeurotic structures; thus, when found in association with breast implants, the fibrous capsule that forms around the implant has been implicated as a possible source.1 There are only 24 cases of implant-associated breast desmoids in the literature. Among these, there are only seven cases following saline implants. There is one case where a patient's silicone implants were replaced with saline implants, and then a desmoid tumor subsequently developed. The remaining 16 cases occurred in association with silicone implants. Among the cases associated with saline implants, one case occurred following implant rupture,2 one case had a concurrent ductal carcinoma before complete surgical resolution,3 one case occurred 3 months after the additional surgical trauma of an unspecified revision procedure,4 and in two cases the implant was inserted after the additional surgical trauma of mastectomy.1,5 Our patient's desmoid developed spontaneously after saline implant augmentation without any of these confounding factors. It is important to note that among all the reported cases of breast desmoids, approximately 10 percent have occurred in patients with breast implants. Is there an association here to be uncovered? There has been very little written regarding a relationship between the biomaterial composition of the prosthesis and the risk of developing a breast desmoid. Because there is evidence that desmoids form after various different types of breast operations (e.g., excisional biopsies, lumpectomies, mastectomies, breast reductions), their development may simply be the result of a cellular transformation in association with postsurgical scarring. However, the possibility of such a transformation occurring because of the presence of foreign biomaterials is a distinct possibility that is a potential topic of further investigation. Interestingly, to date, there are no case reports of a desmoid tumor following purely autologous breast reconstruction. As the number of women electing to undergo breast augmentation increases and more cases are reported in the literature over the upcoming years, an association between breast implants and desmoid tumors, or the lack thereof, may become more apparent. Ziyad S. Hammoudeh, M.D. Department of Surgery, Wayne State University, Detroit, Mich. Vigen B. Darian, M.D. Division of Plastic Surgery, Department of Surgery, Henry Ford West Bloomfield Hospital, West Bloomfield, Mich.

Extensive alveolar-type paranasal sinus and orbit rhabdomyosarcoma with intracranial invasion treated successfully

We report a case of extensive paranasal sinus and orbit rhabdomyosarcoma (RMS) with intra-cranial invasion treated successfully with chemotherapy and radiotherapy. A 13-years-old male patient complained of painless and progressive proptosis of his left eye for two weeks. Ocular examination showed elevated intraocular pressure, limited extraocular movement, proptosis, and conjunctival ciliary injection in the left eye. Brain CT and MRI demonstrated a large enhancing soft tissue mass lesion with bone destruction involving left ethmoid sinus, nasal cavity, maxillary sinus, and orbital cavity with crossing of the midline to the right ethmoid sinus, nasal cavity, and intra-cranial invasion across the frontal base. The pathology of tumor biopsy revealed rhabdomyosarcoma, alveolar type. Systemic survey showed no evidence of distant metastasis. Then, the patient received combined radiochemotherapy with Taiwan Pediatric Oncology Group Rhabdomyosarcoma 2007 High-risk Treatment Protocol. No light perception in his left eye with optic disc atrophy was noted at the beginning of radiotherapy. After 44 weeks of combined radiochemotherapy, the tumor regressed, and no recurrence has been noted until now. In young patients with sudden-onset proptosis, RMS should be considered, and early diagnosis is crucial due to more effective prognosis with current radiochemotherapy protocol.

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 (-)).