Objective: This study describes a case of nodular fasciitis involving the hand and reviews the neoplasm's pertinent clinical, histologic, and pathologic features. Methods: The patient's chart, operative record, histologic specimens, and relevant literature were reviewed. Results: We report a case of nodular fasciitis involving the hand of a 55-year-old woman that was treated with marginal excision. Conclusions: Nodular fasciitis is a self-limited, benign soft tissue tumor composed of fibroblasts and myofibroblasts that typically afflicts younger patients and rarely presents in the hand. Because of its presentation, it can be easily mistaken for a malignant neoplasm. However, most cases represent a reactive and therefore a polyclonal process. Marginal excision generally provides definitive treatment. Nodular fasciitis is a self-limited, benign lesion composed of fibroblasts and myofibroblasts. Because of its rapid growth and worrisome histologic features, it may be mistaken for a sarcoma. This lesion is typically found in the proximal upper limb, however, it may rarely present in the hand.A 52-year-old, right-hand-dominant African American woman presented with a 6-month history of a slowly growing painless mass over the volar aspect of the right little finger metacarpophalangeal (MCP) joint. She denied any history of trauma, infections, foreign travel, or other soft tissue masses. She denied other neurologic symptoms, hand weakness, pains, fever, chills, unexpected weight loss, or other constitutional symptoms. Her medical history was significant for carpal tunnel syndrome, hypertension, iron deficiency anemia, and asthma.Physical examination of the hand revealed a multinodular soft tissue mass, measuring approximately 3 × 4.3 cm, involving the volar aspect over the right little finger MCP joint area. The lesion was firm, nontender, and immobile. No erythema, tenderness, warmth, or fluctuance was present in the hand. Full active and passive flexion of the little finger MCP joint was restricted, limiting opposition as well. Range of motion in the other MCP joints was normal, along with that of all the proximal and distal interphalangeal joints. Neither epitrochlear nor axillary lymphadenopathy was palpable. Sensation was fully intact throughout both hands as determined by Semmes-Weinstein monofilaments.Radiographs of the right hand revealed a noncalcified soft tissue mass adjacent to the ulnar aspect of the little finger proximal phalanx. Magnetic resonance imaging showed a 2.3 × 2.3 × 1.3-cm lobulated, sharply marginated lesion. The distal extent of the tumor lay directly on the volar cortex of the little finger proximal phalanx, whereas the proximal extent began at the level of the MCP joint, without invading either of them. Magnetic resonance imaging demonstrated the mass in contact with both flexor and extensor tendons, without completely encasing them .4Nodular fasciitis is common in young adults, aged 20–40 years. Our patient was significantly outside this age range at 52 years. In fact, only 13% of cases are found in patients older than 50.1The differential diagnosis of nodular fasciitis in the hand includes fibromatosis, fibrosarcoma, fibrous histiocytoma, Dupuytren's nodules, giant cell tumor of the tendon sheath, synovial sarcoma, and calcifying aponeurotic fibroma.11After the history and physical examination , radiographs (which are inexpensive and rapid) should be obtained. However, they generally do not provide detailed anatomic information. Magnetic resonance imaging is useful in determining tumor extent and may help establish a diagnosis. Computed tomographic scans have little utility in evaluating soft tissue masses because they often have similar attenuations as muscle.12Most cases present as subcutaneous masses, but occasionally they can involve fascia and muscle. In rare instances, the mass can grow large enough to extend through the skin. Nodular fasciitis has a characteristic histologic pattern, composed predominantly of plump, immature fibroblasts in an abundance of ground substance. This imparts a loosely textured or feathery appearance. Mitotic figures are common; however, they are never atypical, unlike in sarcomas.3Marginal excision generally provides definitive treatment, with recurrence being rare.In this patient, the mass was found to be strongly adherent to the surrounding structures, in contrast to other case reports that have described a mobile mass that is readily dissected.


Neurofibromatosis type 2 is an inherited autosomal dominant syndrome, characterized by multiple neoplasms of the central and peripheral nervous system associated with ocular abnormalities. The most common tumor associated with the disease is the vestibulocochlear schwannoma , and as many as 10% of patients with this tumor have neurofibromatosis type 2. In this report we aim to present a 34-year-old male who was seen for bilateral hearing loss. During his workup, which included cranial computer tomography, he was found to have multiple intracranial masses. Cranial and whole spine magnetic resonance imaging showed bilateral vestibulocochlear schwannoma, multiple meningiomas, and one intramedullary tumor. Based on clinical and imaging findings the diagnostic of neurofibromatosis type 2 was made. Neurofibromatosis type 2 is a rare disease, with incidence of 1 in 33,000 to 40,000 . It is sThe aim of this report is to present a case of sporadic NF2, in which diagnosis was established based on medical history, clinical symptoms and image findings on magnetic resonance (MR) imaging.A 34-year-old Caucasian Brazilian male was seen at an outside facility for progressive bilateral hearing loss. A head computed tomography (CT) showed multiple intracranial masses. He was then referred to our institution for further work up. Physical examination revealed an alert and oriented patient, with normal cognitive function. He had bilateral hearing loss and diminished visual acuity. Few scattered skin lesions that resembled skin tags were also found. The clinical history included a past of tonic-clonic seizures, treated with fenitoin for a year. Also, a complaint of chronic headache associated with vertigo was identified.He was referred for an otologic evaluation, which included an audiometric exam that showed bilateral sensoneural hearing loss, profound on the right, and moderate on the left. Also, the brainstem evoked response audiometry showed absence of response on the right and only one wave on the left.Review of the brain MR imaging findings showed bilateral acoustic schwannoma, and another four enhancing masses consistent with meningiomas, involving the parafalcine region, sphenoid wing, frontal and parietal lobes Figures  and 2. SAlthough he had no family history, the image findings of bilateral acoustic schwannoma, multiple intracranial meningiomas, and one intramedullary tumor, suggested the diagnosis of NF2.The term MISME has been proposed to the NF 2 syndrome, due to multiple inherited schwannomas (MIS), meningiomas (M) and ependymomas (E). In addition to the neoplasms, posterior subcapsular lenticular opacity  is often present. Patients with NF2 may have cutaneous schwannomas that resemble skin tags, but they rarely have café -au-lait spots and do not demonstrate the cutaneous neurofibromas like NF1. In 1997 Gutmann et al. proposed revised criteria for diagnostic of the syndrome [The diagnosis of NF2 is usually made in the second or third decade of life, with a peak in the 20s. The clinical presentation of NF2 varies, but approximately a range from 30-45% of patients are diagnosed because of symptoms resulting from cranial nerve (CN) VIII schwannomas, such as hearing loss, tinnitus, balance impairment, and weakness in CN VII distribution. The reason for this is that CN VIII schwannomas are symptomatic at a relatively small size. The tumor causes symptoms by compressing or stretching the cochlear nerve, compressing the blood supply to the nerve or to the cochlea, or causing hemorrhage into the nerve or cochlea . In MautSeveral authors have studied series of cases to try to define incidence of the tumors in cases of NF2. Mautner et al studied 48 patients with NF2, in which the prevalence of findings were: vestibular schwannomas (CN VIII) in 46 96%), spinal tumors in 43 (90%), posterior subcapsular cataracts in 30 (63%), meningiomas in 28 (58%), and trigeminal schwannomas in 14 (29%) 6%, spina reportedThe majority of intramedullary spine tumors in NF2 is ependymomas and arises in either the upper cervical cord or the conus. Meningiomas present as intradural extramedullary neoplasms that are very similar to spontaneous meningiomas and most commonly involve the thoracic spine; frequently they are multiple in number. Patronas et al  studied CN: Cranial nerve; CT: Computed tomography; MISME: Multiple inherited schwannomas, meningiomas and ependymomas; MR: Magnetic resonance; NF2: Neurofibromatosis type 2.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GS conceived the study. RFC, TCT, IGV and RRB performed the literature review. GS, ELG, and EM edit and coordinated the manuscript. All authors read and approved the final manuscript.


Although tumors on the surface of the skin are considered to be easily recognizable, neglected advanced skin neoplasms are encountered even in the 21st century. There can be numerous causes of the delay in the diagnosis: fear of the diagnosis and the treatment, becoming accustomed to a slowly growing tumor, old age, a low social milieu, and an inadequate hygienic culture are among the factors leading some people not to seek medical advice. The treatment of such advanced neoplasms is usually challenging. The therapy of neglected cases demands an individual multidisciplinary approach and teamwork. Basal cell carcinoma (BCC), the most common cutaneous tumor, usually develops in the elderly, grows slowly, and has an extremely low metastatic potential; these factors are suggesting that BCCs might well be the “ideal candidates” for neglected tumors. Five neglected advanced cases of BCC were diagnosed in our dermatological institute between 2000 and 2009. The clinical characteristics and treatment modalities of these neoplasms are discussed, together with the possible causes of the neglect. Tumors on the surface of the skin are generally visible and considered to be easily recognizable both for health-care professionals and for the patients themselves. However, people with neglected advanced skin neoplasms are still encountered in dermatological practice in the 21st century. There can be numerous causes of the delay in the diagnosis: the person may fear the diagnosis and the treatment or become accustomed to the usually slowly-growing tumor. Old age, a low social milieu, and an inadequate hygienic culture may also be factors explaining why some people are not aware of the significance of a delayed diagnosis. Basal cell carcinoma (BCC) is the most common cutaneous tumor and one of the most frequent skin diseases observed by dermatologists , 2. MostThe main clinical characteristics of the patients and the treatment applied are listed in When a 44-year-old male first sought for medical advice in December 2009, he had already had a mutilating, horrifying tumor on the right side of his face for 10 months. He lived alone in a farmhouse in a rural, but quite densely populated area. Two years previously, his own dog had clawed on the right side of the patient's nose, with the injury leaving a scar. Eleven months before his presentation, he had suffered another minor injury in the same region caused by a splinter of wood. A gradually growing, ulcerating lesion had subsequently developed on his face . The cliA 96-year-old female, living in a small town, presented in November 2007 with a 2-year history of a growing tumorous mass in the right inguinal region. The tumor was removed and the histopathology confirmed the presence of squamous cell carcinoma (SCC). At the same time, an ulcerated tumor on the right parieto-occipital region of her head was diagnosed clinically as BCC. Excision or irradiation therapy was planned after the removal of the inguinal tumor, but the patient did not continue the treatment. In 2009, she received neurosurgical treatment and was then referred to dermatology because of her growing cranial tumor . An inciA 63-year-old man living in an agricultural town had had a small exophytic skin-colored nodule in the middle of his back for many years. This had started to grow rapidly and become ulcerated during the past few months. By his first medical visit in September 2009, suppurative inflammation with abscess formation had developed in the nearby dermis . ConsequAn 84-year-old male resident in an agricultural village presented in September 2008 with numerous actinic keratoses on his head and neck and with a slowly growing tumorous mass in the presternal area, which he had observed one year previously . The tumA 68-year-old male, living in a village, underwent renal transplantation in 1995 and had subsequently received immunosuppressive therapy. A slowly growing tumor developed on the right side of his neck one year prior to his clinical presentation in 2009. Both the clinical and the histopathological diagnosis indicated BCC. Additionally, there were two small SCCs in the preauricular region and on the right ear .Around one-third of giant BCCs result from patients' neglect , 2, 5. TIt is interesting, that although the patients themselves are more or less aware of the growing tumor, it is usually some external event that finally impels them to seek medical advice, for example, a sudden change in the lesion (bleeding or sudden growth) or encouragement by another person (a family member or friend) –3, 6, 7.Another challenging question is the treatment of these advanced neoplasms. Numerous possibilities are available for ordinary BCCs: surgical excision, Mohs micrographic surgery, PDT therapy, cryosurgery, immunotherapy, and radiotherapy are the most frequently used techniques , 3. The Neglected advanced skin tumors can be encountered even in the 21st century. BCC, the most common cutaneous tumor, usually develops in the elderly, grows slowly, and has an extremely low metastatic potential making it an “ideal candidate” for a neglected tumor. Although there are many possibilities for the treatment of BCCs, the therapy of such neglected cases always demands an individual and multidisciplinary approach and teamwork.


The availability of technological advances like ultrasonography (USG) and magnetic resonance imaging (MRI) has made the diagnosis of rudimentary horn pregnancy possible at an early gestation. However, in advanced pregnancy, such cases can sometimes pose a diagnostic dilemma and are recognized only when patient presents with abdominal pain and collapse and is taken for laparotomy. We report one such rare case of a nulliparous female who was carrying on well with her pregnancy till she developed symptoms of acute abdomen at 28 weeks of gestation. She underwent USG and MRI but it was only after laparotomy that a final diagnosis of a pregnancy in a rudimentary horn with placenta percreta perforating through the fundus could be made. There was a significant amount of hemoperitoneum; however, the horn was intact and the fetus could be salvaged. We excised the rudimentary horn with ipsilateral tube and ovary. Post operatively, both the mother and the baby were discharged in healthy condition. Pregnancy in a non-communicating uterine horn culminating in the delivery of a live fetus is a rare case. These pregnancies hardly reach viability and often result in rupture of the horn in second trimester. In our case though the patient presented with signs of hemoperitoneum at seven months of pregnancy, the cause was placenta percreta perforating the fundus of an intact horn and timely laparotomy saved both mother and fetus.A 21-year-old nulliparous lady was referred to our emergency by a general practitioner at 28 weeks of gestation with history of abdominal pain since one week. There was no history of any discharge per vaginum, loss of fetal movements, abdominal trauma, or any bladder or bowel disturbance.She was gravida two with one spontaneous abortion at 3 months of gestation about one year back, for which a curettage was done. Rest of her medical and surgical history was unremarkable. Her present pregnancy had been uneventful till now and her early pregnancy scans were reported to be normal.On admission, her general condition was poor, and there were signs of hypotension and tachycardia. Her upper abdomen was soft and non tender while there was guarding and tenderness in lower abdomen. Her uterus was 26 weeks gravid, with contour well made out. Fetal parts were made out with difficulty. The flanks were dull on percussion. Fetal heart sounds could not be auscultated. On per speculum examination, there was no bleeding. Her vaginal examination revealed a soft cervix with os closed, and a firm round nontender 4×4cm mass in the anterior fornix toward the right side of cervix.The patient had undergone an magnetic resonance imaging (MRI) four days back as advised by the treating physician before presenting to this hospital. The MRI reported a didelphys uterus with a single fetus in left uterine body. The placenta was left lateral and there was no free fluid in abdomen . It alsoPreoperatively, there was hemoperitoneum of about 1.51. There was an enlarged gravid intact rudimentary horn and brisk bleeding was seen from the prominent blood vessels scattered all over its fundus . The horHer postoperative course was uneventful. Pathological evaluation of the specimen confirmed the presence of placenta percreta invading the serosal layer. Microscopic examination confirmed the lack of any communication in the fibrous band connecting the rudimentary horn with the uterus. The neonate was discharged from the hospital after one and a half months in a healthy condition after gaining weight up to 1400 g.Pregnancy in a noncommunicating rudimentary horn has a reported incidence of 1 in 1,00,000 to 1 in 1,40,000. It is ex3As rudimentary horn pregnancies are always associated with catastrophic outcome, every effort should be made to diagnose them at an early gestation. A detailed history should be taken in every patient on her first visit including any complaints of severe dysmenorrhea. However, the rudimentary horn may be underdeveloped and its endometrium non functional and dysmenorrhoea may be absent as seen in our case. A careful pelvic examination in the first trimester showing deviated uterus with a palpable adnexal mass should arouse suspicion of a mullerian anomaly. It can be confirmed by an ultrasound examination though the sensitivity remains only 26%. The enlaRudimentary horn pregnancy can be further complicated by placenta percreta due to the poorly developed musculature, scant decidualization and small size of the horn; the reported incidence being 11.9%. MRI has Thus we conclude that high clinical suspicion, early diagnosis and timely laparotomy can reduce the perinatal mortality for both mother and fetus. When diagnosed early, excision of rudimentary horn with ipsilateral salpingectomy is the recommended surgical treatment and provides the best prognosis.


Less than 80 reported cases of paragangliomas of the larynx are reported in the literature. A role for external beam radiation in this disease has not yet been explored. We present four cases of laryngeal paragangliomas treated at a large tertiary-care cancer center over a 35-year period.124 cases of head and neck paragangliomas treated at a single institution from 1970 to 2005 were retrospectively studied. Patients with laryngeal paragangliomas were identified, and a comprehensive clinico-pathological review was undertaken.We identified 4 patients with tumors arising in the larynx at the following subsites: supraglottis (2), glottis (1), and subglottis (1). Three patients were treated with surgery and one with definitive radiation alone.Laryngeal paragangliomas are rare tumors and are adequately treated with surgical resection. We also present one patient who was treated with radiation and had disease stabilization. Accurate histological classification is critical, and the role of genetic testing is emerging. Paragangliomas of the laryngopharynx are rare tumors that are of neuroendocrine origin and arise from the neural crest-derived cells of the parasympathetic nervous system. Since its first description in the literature in 1955, fewer than 80 such cases have been reported . Since tAll laryngeal paragangliomas treated at the University of Texas M.D. Anderson Cancer Center over the last 35 years were reviewed after IRB approval was obtained. We identified 124 patients with cervical paragangliomas, five of whom had tumors localized to the laryngopharynx. One patient presented to M.D. Anderson for a second opinion for a hypopharyngeal paraganglioma. This patient was eliminated from our study as he was lost to follow-up. Pathology reports, operative notes, video stroboscopic films and reports, radiologic imaging, and all clinical notes were reviewed for the remaining patients. A comprehensive review of the literature was also performed.A 67-year-old woman presented with voice changes. On flexible endoscopy, a submucosal mass was seen extending through the glottic aperture. The patient underwent an incisional biopsy, which revealed a paraganglioma by immunohistochemical and histopathological criteria. The patient was taken for surgery, and a direct laryngoscopy revealed a tumor originating in the subglottis, extending to the vocal folds at the anterior commissure. Due to the large pushing borders of the tumor that caused extensive damage to the thyroid and cricoid cartilage, a total laryngectomy was performed. Cartilage destruction with no apparent invasion of the larynx from the paraganglioma was evident on histologic evaluation. The patient remained free of disease and deceased from other causes two years later.A-50 year-old man presented with a recurrent mass of the left supraglottic larynx that had been treated with a supraglottic laryngectomy eight months prior to presentation at our institution. A review of the pathology revealed a paraganglioma. He was treated with a total laryngectomy, but eight months after surgery, he presented with histologically-confirmed bilateral lung metastasis and brain metastasis. He was treated with systemic chemotherapy but ultimately developed spinal metastases and progressive pulmonary disease. The patient ultimately died from his disease 16 months after diagnosis.An 85-year-old woman presented to an outside institution with a two-year history of cough. A supraglottic lesion was noted on axial imaging of the neck, and a biopsy revealed a spindle cell neoplasm. This lesion was followed for one year, but the patient presented to our institution with progression of her disease, which included worsening of cough, new onset of hoarseness, and dysphasia with symptoms of aspiration. On flexible laryngoscopy, the patient had a right laryngeal mass emanating from the right false cord and extended to the aryepiglottic fold and epiglottis Figure . AlthougA 39-year-old black female was noted to have an asymptomatic glottic lesion at the time of a laparoscopic cholecystectomy for cholelithiasis. Intubation was performed without incident, though approximately one week later, she developed shortness of breath and airway compromise. A tracheostomy was placed at an outside facility. She subsequently underwent a panendoscopy and biopsy. On laryngoscopy, the lesion extended from the posterior aspect of the left arytenoid to along the aryepiglottic fold and false vocal cord onto the epiglottis Figure . On CAT A report in 1994 by Ferlito et al critically evaluated all of the reported paragangliomas of the larynx and found that only 62 of these cases met their criteria for this diagnosis . Since tThe management of laryngeal paraganglioma is usually surgical, and it often entails total laryngectomy. In selected cases, conservation surgical approaches have been suggested for oncologic control. Partial laryngectomy is a reasonable option in selected cases, particularly due to low likelihood of regional and distant metastasis ,6. VarieA total of 23 inferior laryngeal paragangliomas have been reported in the literature, 11 of which presented in the subglottis. The other 12 presented as thyroid masses. Two patients with subglottic tumors required total laryngectomy, two required airway reconstruction, and one patient had recurrence . These lAlthough paragangliomas are generally benign tumors , malignaMalignant paragangliomas more commonly metastasize distantly and rarely spread to the lateral neck lymph nodes. Thus, an elective neck dissection in a No neck with a biopsy confirmed malignant laryngeal paraganglioma is generally not advocated ,2. In ouAs previously observed by multiple groups, neither radiation nor chemotherapy has been used successfully as an alternative to surgery in the management of laryngeal paragangliomas ,5,13,14.VHL and NF1 genes are also associated with inherited paragangliomas. In particular, patients with SDHB mutations are at higher risk for developing malignant paragangliomas [The importance of genetic testing for mutations in the SHDB, SDHC, and SHDD genes has recently been highlighted . Mutationgliomas . To datengliomas .Laryngopharyngeal paragangliomas are uncommon tumors that are conventionally treated with surgical resection for oncologic control. Close collaboration with an experienced pathologist is necessary to establish the diagnosis with immunohistochemical evaluation and electron microscopy to confirm the diagnosis. The evolving role of genetic testing is not defined, but it is likely that these four reported cases are sporadic. Surgical resection remains the standard of care for their treatment, although radiotherapy may be considered in selected patients. Close observation with serial imaging is necessary if this treatment approach is undertaken. Although malignant paragangliomas of the larynx have been reported, they are rare and metastasis should raise the question of misdiagnosis. Multi-institutional collaboration may provide greater insight into the true biological behavior of this disease and help optimize treatment approaches.Written informed consent was obtained from the patients for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JRS carried out the data collection and drafted the manuscript. EYH assisted in the concept of the design and editing the manuscript. MDW participated in the data collection. MEK conceived the study design, and helped in drafting and editing the manuscript. All authors read and approved the final manuscript.


Cancers of the male urethra constitute less than 1% of all malignant urological tumours, and the occurrence of sarcomas in the urethra is even less frequent. To our knowledge, only one case has been previously described in the English literature.We report the clinical features, histology, imaging and treatment of urethral leiomyosarcoma in a male patient.The occurrence of sarcoma in the urethra is most unusual, and its appearance as a primary growth in the male urethra is not recorded in the English literature. In conclusion, this case highlights a rare type of primary male urethral malignancy that features a poor prognosis. Primary sarcoma of the urethra in the male patient is extremely rare. Malignant neoplasms of the urethra are typically squamous cell carcinoma. Leiomyosarcoma is a very rare neoplasm of the urinary system with less than 20 cases being reported in the literature up to date.We report below the case of a male patient with ureteral leiomyosarcoma.A 65-year-old Moroccan male patient had undergone a perineal urethrostomy to treat an extended distal urethral stricture. After one year, he presented with 3-month history of dysuria, pollakiuria and urethral bleeding. About six weeks previous to examination, slight terminal hematuria was noticed. His weight was stable and he appeared well nourished. Physical examination of the genitals revealed a firm and non-fluctuant mass embracing the perineum, the proximal part of the penis and the upper part of the scrotum Figure . The proLeiomyosarcoma is a rare soft-tissue tumor that accounts for 10-20% of soft-tissue sarcoma. It is generally seen in middle-aged patients, and affects women more frequently than men . If anatThe authors declare that they have no competing interests.YA analyzed and interpreted the patient data regarding the retroperitoneal disease. MT and ET have made contributions to conception and design, and acquisition of data. AB performed the histological examination of the kidney, and was a major contributor in writing the manuscript. ME have been involved in drafting the manuscript and revising it critically for important intellectual content. MF have given final approval of the version to be published. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Background. Spindle cell lipoma (SCL) is a benign lipomatous tumour, typically occurring in the posterior neck, shoulder or upper back of elderly males. They compose of fat, CD34 positive spindle cells, and ropey collagen on a myxoid matrix. This case highlights a rare presentation of SCL and the need for pre-operative diagnosis. Case Report. A 63-year-old gentleman presented with a pre-existing left supraclavicular mass that had recently increased in size. FNA and CT Scans were performed and results discussed in the mutidisciplinary team meeting. Excisional biopsy was recommended. Radiology. CT neck showed a left supraclavicular mass of fatty density with fine internal septations. A low-grade liposarcoma could not be excluded. Histopathology. FNA was indeterminate. Histology of specimen showed bland spindle cells with no evidence of malignancy. Immuno-histochemistry showed SCL with CD34 positivity and negative staining on CDK4 and p16. Management. Excision biopsy of the mass was performed which was technically difficult as the mass invaginated around the brachial plexus. The patient recovered well post-operatively with no neurological deficits. Conclusion. Spindle cell lipoma is a rare benign tumour and a pre-operative diagnosis based on the clinical context, imaging and immuno-histochemistry is crucial to management. Lipomasare slow growing fatty tumours of mesenchymal origin and can occur in any organ of the body. Spindlecell lipomas (SCLs) are lipoma variants which have the potential of neuromuscular invasion despite their benign histopathology.  This case report highlights a spindle cell lipoma variant in the supraclavicular area found intraoperatively to invade both the paravertebral muscles and the brachial plexus. Computerised tomography (CT) and Magnetic Resonance Imaging (MRI) findings were suggestive of possible malignancy but the biopsy and immune-histochemical stains confirmed the diagnosis as SCL.  This report highlights the need for making a preoperative diagnosis of spindle cell lipoma. This ensures accurate patient consent and appropriate preoperative surgical planning including surgical approach and extent of surgery.A-sixty-three-year-old gentleman presented with a two-year history of a left supraclavicular mass which had increased in size over the previous three months prior to presentation. The swelling caused discomfort with movement but there were no neurological deficits. He was a smoker with a past medical history of hypertension and type 2 diabetes mellitus.  On examination, there was a large tennis ball sized swelling in the left neck level V area with no palpable cervical lymphadenopathy. This swelling was smooth and nontender on palpation. ENT examination was normal.  Fine Needle Aspiration (FNA) of the mass was performed but it was nondiagnostic. CT neck showed a 46 × 69 × 91 mm left supraclavicular mass posterior to the sternocleidomastoid muscle and superficial to the paravertebral muscles, of fatty density with fine internal septations Figures and 2. Th The case was discussed at the multidisciplinary meeting and due to the suspicion of malignancy, and a nondiagnostic FNA, excision biopsy was recommended. The operation was technically difficult because the mass invaginated around the brachial plexus and extended posteriorly through the prevertebral fascia. Complete excision of the mass was undertaken with mobilisation and preservation of the brachial plexus. The patient made a full recovery with no neurological deficits.The specimen weighed 132  grams and consisted of well-circumscribed fatty tissue. Histological examination showed mature adipocytes interspersed with fibrous septa containing bland spindle cells with focal pseudo-angiomatous arrangement. There was the presence of ropey collagen, a very characteristic finding in spindle cell lipoma. No lipoblasts were seen and the spindle cells showed strong uniform immuno-histochemical positivity for CD34.  Subsequent review also showed negative staining for CDK4 and p16, supporting the diagnosis of spindle cell lipoma, and excluding atypical lipomatous neoplasm.Spindle cell lipoma (SCL) is a benign tumour which typically occurs on the posterior neck, shoulder, or upper back in predominantly elderly males. There is a marked male predilection especially in the sixth decade which may be partly explained by androgen receptors reactivity . Other r Histologically it consists of a proliferation of mature adipocytes, collagen fibres, and spindle cells within a myxoid stroma , and 7 aDifferential diagnoses include atypical lipomatous tumour/well-differentiated liposarcoma, pleomorphic lipoma, neurofibroma, nuchal fibroma, lipoblastoma, hibernoma, cellular angiofibroma , extrama It is important to note that reactivity to CD34 immuno-histochemical stain is not specific to only spindle cell lipoma as other entities can demonstrate immunoreactivity to CD34 such as atypical lipomatous neoplasm (ALN)/well-differentiated liposarcoma (WDL).  ALN/WDL is the most common form of liposarcoma seen in late adult life with equal gender affectation generally. They contain a significant component of mature fat and tend to have less well-defined borders than lipomas . The fav SCL shows cytogenetic aberrations—mostly loss of 16q material and less frequently material from 13q. This coupled with the usual absence of giant marker and ring chromosomes that are typically seen in atypical lipomatous tumour/well-differentiated liposarcoma supports their distinction.  Immunostaining for MDM2 and CDK4 has been shown to be a relatively sensitive and specific means of identifying and separating atypical lipomatous neoplasm/well-differentiated liposarcoma from various benign lipomatous lesions , 16.  In this case report, histologic findings did not demonstrate sufficient nuclear atypia to suggest a diagnosis of atypical lipomatous tumour and further immuno-histochemical markers that would make such a diagnosis possible  were found to be negative.  SCL shares overlapping clinical, morphologic, and immuno-histochemical findings with pleomorphic lipoma as well as the same cytogenetic aberrations. The differential diagnosis of SCL or pleomorphic lipoma depends on which elements predominate .  Dermatofibrosarcoma protuberans (DFSP) is also a differential of SCL. It however typically arises in the dermis, often in younger patients and lacks the characteristic ropey collagen of SCL .  Schwannoma and neurofibroma have a similar striking nuclear palisading and conspicuous mast cell infiltrate present in some SCL. These however invariably express S-100 protein which is negative in SCL .  SCL may also be mistaken for several sarcomas, including myxoid liposarcoma or spindle cell liposarcoma. SCL is however more circumscribed, lacks lipoblasts, with characteristic ropey collagen bundles. The presence of multinucleated floret-like giant cells is characteristic of pleomorphic lipoma and is occasionally seen in atypical lipomatous tumour/well-differentiated liposarcoma.  In this case report, the spindle cell lipoma presented a diagnostic and management dilemma as it could not be clinically differentiated from a liposarcoma which would require a more radical approach to treatment. The radiologic findings of a complex internal architecture with fine septations allowed differentiation of the preoperative differential diagnosis from a benign lipoma or possible malignancy but could not differentiate between a liposarcoma or a spindle cell lipoma.  The authors suggest that when radiologic findings suggest a complex internal architecture suspicious for a diagnosis of a possible lipomatous tumour, it is imperative to obtain a biopsy prior to definitive surgery. Histologic interpretation is crucial and this can be supported by the use of immune-histochemical stains. Immuno-histochemical stains demonstrating positivity for CD34 can be helpful in diagnosis of CD34 positive tumours such as spindle cell lipoma, and the differential diagnoses discussed such as atypical lipomatous tumour and dermatofibrosarcoma protuberans (DFSP). As discussed, further differentiation can be made based on clinical presentation, immunoreactivity for actin or desmin, S-100 protein staining of mature lipocytes, the presence/absence of ropey collagen bundles, multinucleated floret-like giant cells and cytogenetic aberrations. In difficult cases, fluorescence in situ hybridization (FISH) analysis can be helpful. A preoperative diagnosis of spindle cell lipoma is crucial to surgical management as it impacts on the consent process, surgical approach, extent of surgery, and management.  In making a preoperative diagnosis, tissue sampling is required with either FNA or core biopsy. However, it is important to note that FNA may be nondiagnostic as it was in this case, because the specimen consisted largely of fatty cells. Core biopsy is preferable and can be obtained by an initial procedure under anaesthesia prior to definitive surgery based on a histological diagnosis. A preoperative diagnosis will guide the consent process as such patients can be warned of the risks of neurovascular injury as well as determining the extent of surgery.


Situs inversus is a congenital anomaly characterized by the transposition of the abdominal viscera. When associated with dextrocardia, it is known as situs inversus totalis. This condition is rare and can be a diagnostic problem when associated with appendicular peritonitis.situs inversus at the left side of his liver, and his appendix was perforated in its middle third. A complementary post-operative thoracic and abdominal tomodensitometry revealed a situs inversus totalis.We report the case of a 20-year-old African man who presented to the emergency department with a 4-day history of diffuse abdominal pain, which began in his left iliac region and hypogastrium. After examination, we initiated a surgical exploration for peritonitis. We discovered a situs inversus is a rare association that can present a diagnostic problem. Morphologic exploration methods such as ultrasonography, tomodensitometry, magnetic resonance imaging, and laparoscopy may contribute to the early management of the disease and give guidance in choosing the most appropriate treatment for patients.Appendicular peritonitis in  Situs inversus is a congenital anomaly characterized by the transposition of the abdominal viscera. It may or may not be associated with dextrocardia, also known as situs inversus totalis [situs inversus discovered in relation to the treatment of generalized acute peritonitis of appendicular origin. This case is particularly interesting because of the scarcity of this association and the diagnostic difficulties that may arise because of unusual symptoms. totalis ,2. GenerA 20-year-old African man presented to the emergency department at the Aristide Le Dantec hospital with 4-day history of diffuse abdominal pain in his left iliac region and hypogastrium. This pain was associated with bilious vomiting and fever. On examination, he was found to be in a good general condition. He had a fever at 40°C, a pulse rate of 120/minute, and blood pressure of 120/70 mm Hg. His physical examination revealed a generalized abdominal tenderness predominantly over his left lower and hypogastric quadrants.3 with 93% neutrophils, 42% hematocrit, and platelets at 323,000/mm3. An X-ray of our patient's abdomen showed small bowel loops and a diffuse grayness. After a pre-operative reanimation, a median laparotomy was performed. The exploration showed an acute generalized peritonitis with 300 mm3 of pus, false membranes, situs inversus (Figure Laboratory investigations showed that he had a white blood cell count of 18,900/mmsitus inversus totalis with dextrocardia and a left-sided liver (Figures Bacteroides fragilis sensitive to the combination of amoxicillin and clavulanic acid. Surgical pathology confirmed acute appendicitis with suppurative necrosis of his serous membrane. No post-operative complication was noted, and he was discharged home eight days after his operation.A post-operative abdominal tomodensitometry with a frontal view of our patient's abdomen and lower chest was performed to assess his condition. This revealed a  Figures  and 4. ASitus inversus is a positional anomaly that rotates the abdominal internal viscera. It is known as situs inversus totalis when it is associated with a transposition of the thoracic organs. Situs inversus is a rare congenital anomaly with an incidence in the population of only 0.001% to 0.01% [to 0.01%  with a mto 0.01% . Its trato 0.01% .Situs inversus results from a rotation in the opposite direction of the viscera during the development of the embryo [situs inversus may face diagnostic problems because of the unusual localizations of their symptoms. In the case of our patient's pain in the left iliac fossa, the differential diagnosis we made was extensive. Even in patients without situs inversus, the right iliac appendicular symptoms would be found in only 60% of cases [situs inversus may be due to an abnormally long appendix projected to the left, or to intestinal hyperkinesis.e embryo ,3. Patieof cases ,3. The psitus inversus and 0.016% with situs inversus totalis [situs inversus were reported in the literature [situs inversus [situs inversus is not automatically evoked, which delays the appropriate management of patients. As a consequence, as in the case of our patient, peritoneal diffusion may eventually develop.A study of 71,000 patients appendicular symptoms found that 0.04% of cases involved left iliac localization, comprising 0.024% with abdominal  totalis ,4. Untilterature . Half ofsitus inversus with acute appendicitis. Medical imaging can help clinicians to arrive at a correct diagnosis. Abdominal X-ray, ultrasonography, and tomodensitometry can also facilitate an accurate and early diagnosis if a patient is unaware of this positional anomaly [Meanwhile, the usual differential diagnosis of left lower quadrant abdominal pain in an adult man includes, among others, sigmoid diverticulitis, epididymitis, bowel obstruction, psoas abscess, and, in this rare instance,  anomaly ,3,4. Med anomaly .situs inversus is very rare. Very few cases have been reported in the literature. This condition poses a diagnostic problem that can be decreased by including morphologic exploration methods such as ultrasonography, tomodensitometry, and laparoscopy. These procedures allow the early management of the disease and guide therapeutic choices.The occurrence of appendicitis with The authors declare that they have no competing interests.MC and AOT performed the surgical procedure and drafted the case report. IK and OK interpreted and analyzed the tomodensitometry findings. MD participated in the diagnostic and therapeutic decisions. AD and CTT made major contributions to writing the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from our patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Wireless capsule endoscopy is an important tool for minimally invasive evaluation of the small bowel, allowing improved diagnostic yield with low complication rates relative to traditional modalities. Recently however, reports on small bowel perforation after wireless capsule endoscopy have surfaced. Here we present the first case of acute small bowel perforation in a middle-aged male in the United States.A 58-year-old male with a presumed quiescent history of Crohn’s Disease presented to the Emergency Department in a septic state 48 hours after a wireless capsule endoscopy procedure complaining of abdominal pain, distension, and frequent emesis. A computed tomography scan of the abdomen was suggestive of small bowel perforation and ischemic enteritis. The patient was adequately resuscitated and taken to the operating room for an ileocecectomy and extensive resection of the small bowel. Pathology of the resected specimen revealed an ileal stricture and associated necrotizing ileitis, and a perforation just proximal to the stricture.Wireless capsule endoscopy remains the preferred endoscopic imaging method of the small bowel. This case illustrates the importance of appropriate patient selection and evaluation of functional patency of the small bowel prior to wireless capsule endoscopy, especially with the growing role of this procedure in the evaluation of inflammatory bowel disease. Since its conception in 2000, wireless capsule endoscopy (WCE) has become an established tool for minimally invasive evaluation of the small bowel (SB). Today WCE is most commonly utilized for evaluation of obscure GI bleeding unidentified by traditional endoscopic techniques . In receWhile WCE is a generally safe and well tolerated procedure in patients with IBD, it does carry the potential for serious complications. Capsule retention is a well recognized complication occurring at a higher frequency in patients with known or suspected CD . CapsuleA 58-year-old Caucasian male with a reported 30 year history of CD presented to his gastroenterologist with intermittent diarrhea controlled by bismuth subsalicylate (Kaopectate). He denied any recent flares or pharmacologic management. A colonoscopy was negative for classical findings of CD and the patient next underwent WCE to document possible disease in the SB. On the evening of the procedure the patient reported poorly localized abdominal pain and copious non-bilious/non-bloody emesis. Over the next 48 hours he experienced progressive abdominal distention and presented to the Emergency Department. He denied the chronic use of NSAIDs or a history of prior abdominal operations.9/L (4.5-11.0 × 109/L) and platelet count of 79 × 109/L (150-450 × 109/L). His creatinine was 203.3 umol/L (58-111 umol/L) and total bilirubin was 171 umol/L (3-22 umol/L). A CT scan of the abdomen/pelvis revealed a capsule endoscope located near the terminal ileum along with diffusely thickened bowel wall, fat stranding in the right lower quadrant, hemorrhagic ascities and loculated free air  patch and loose suture. He required 12 subsequent operations for various complications and was discharged after 1 month with an ileostomy and colostomy. Pathology of the resected specimen showed an ileal stricture 15 cm proximal to the ileocecal valve, with associated necrotizing ileitis. A perforation was noted just proximal to the stricture where the capsule endoscope was found.WCE is a safe and well tolerated procedure with few complications. Over 400,000 capsules have been deployed worldwide since 2000 with rare complications and no reported deaths . To dateDespite concerns over capsule retention and possible obstruction, the widespread use of WCE for evaluation of CD is justified by improvements in diagnostic yield relative to traditional modalities, with low procedure risk. Overall diagnostic yield has commonly been reported between 15% and 44%, and a recent case series has shown a diagnostic yield of 86% leading to a change in management in 64% of cases ,12.In the present case, capsule impaction secondary to a stricture in the terminal ileum was likely the inciting mechanism leading to acute SB obstruction and subsequent perforation. The use of more rigorous pre-procedure evaluation in patients with suspected or known CD should therefore be pursued. Specifically, additional imaging in the form of barium follow-through, and CT/MR enterography may help characterize SB disease, although the presence or absence of a stricture does not necessarily preclude the possibility of SB obstruction. In this regard, the patency capsule has shown promise for the evaluation of patients with known strictures or other SB disease leading to obstruction . Spada eThe advent of WCE has revolutionized imaging of the SB, improving diagnostic yield at relatively low complication rates. Our report, in combination with the Repici and Um reports, suggests that more serious complications than previously anticipated exist for this technique and may not be limited to the elderly. Furthermore, SB perforation should be recognized as a possible complication in patients with well established, suspected, or even a presumed quiescent history of CD. Capsule endoscopists must exercise extended caution in patient selection, especially as utilization of WCE increases in the management of IBD.


The most common tumours of the adrenal gland are adenoma, pheochromocytoma, adrenocortical carcinoma, and metastases. Although the imaging features of these tumours are established, the imaging characteristics of uncommon adrenal masses are less well known. In patients with extradrenal tumour, incidental discovery of an adrenal mass necessitates excluding the possibility of metastatic malignancy.A 52 year-old female was diagnosed with oesophageal adenocarcinoma and treated with oesophagectomy and adjuvant chemotherapy. Sixteen months later on staging CT scan a 2 × 2 cm adrenal mass was detected, which increased in size over a period of time to 3 × 3 cm in size. Adrenalectomy was performed and histological examination revealed metastatic adenocarcinoma within an adrenal adenoma.The present case highlights the unusual behaviour of an oesophageal adenocarcinoma causing metastasis to an adrenocortical adenoma. Due to improvements in radiological diagnosis capabilities, there has been a recent increase in adrenal tumours that are incidentally discovered in patients who underwent medical examination by ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI), i.e. "adrenal incidentalomas" . In patiA 52-year-old woman presented with complaints of dysphagia and hoarseness of voice in December 2003. She was diagnosed to have an advanced moderately differentiated oesophageal adenocarcinoma for which adjuvant chemotherapy was started. In May 2004, she underwent a transcervical oesophagectomy and gastric pull up followed by postoperative chemotherapy. The postoperative interval was uneventful. Staging CT scan performed in April 2005 revealed a 2 × 2 cm left adrenal mass. Half body positron emission tomography (PET) scan with 18F-2-fluoro-D-deoxyglucose (FDG) with CT anatomical image fusion performed in April 2005 and again in February 2006 revealed an increasing left adrenal mass measuring 3 × 3 cm in size. The left adrenal gland showed an intense abnormal FDG uptake  declare that they have no competing interests.IB- wrote the draft manuscript and did the literature search.GC- performed the radiology and contributed to drafting the manuscript.SM- performed the surgery and contributed to drafting the manuscript.AW- performed the pathology, diagnosed the condition and prepared the photomicrograph.All authors read and approved the final version of the manuscript.AcknowledgementThe authors are grateful to Lee Gumble for his assistance in photography.


Myxoid Neurothekeoma is a rare benign nerve sheath tumor, commonly seen in young females. Patients usually present with a small nodule in different anatomical sites, commonly involving the face and the upper limb. We present the case of a five-month-old boy, who presented with a nodule on the left thumb. Punch biopsy and immunostaining confirmed the diagnosis of myxoid neurothekeoma. We believe this is the first reported case of myxoid neurothekeoma below 12 months of age. Neurothekeoma is a rare benign soft tissue tumor with fairly distinctive histological features. It is commonly located on the upper extremities or the head and the neck. HistologComplete excision is the mainstay of treatment for neurothekeoma. Recurrence is attributed to incomplete excision. We present the first case report of rare myxoid neurothekeoma in an infant of 5 months’ age and discuss the histopathological features distinguishing it.2, pinkish red, firm, immobile, non tender, non fluctuant intradermal swelling, with intact overlying skin [A five-month-old boy was brought with left thumb swelling of three weeks’ duration. It was a 0.5 × 0.5cming skin . There wing skin .Soft tissue tumors are uncommon in the pediatric age group and differ from adult incidences in different aspects, including frequency, anatomical site and prognosis. Differential diagnosis of the dermal nodule in infancy and young adulthood should include fibrous tumors, histiocytic tumors, lymphocytic tumors, melanocytic tumors and neural tumors.Neurothekeoma is a rare benign tumor of the nerve sheath, with a distinct histomorphological character. It was described initially by Hakin and Reed, in 1969, as Nerve sheath myxoma. The term Neurothekeoma was described by Gallager and Helwing, in the 1980s. There isThis disease is commonly present as small solitary nodules on the face and the upper limb; however, other anatomical sites have been reported, such as oral cavity, cauda equina, shoulder and neck.Based on histomorphological appearance and immunohistochemical findings, there are three variants of neurothekeoma–myxoid , cellular and mixed type.The myxoid type is characterized by greater degree of myxomatous changes, less cellularity with well-circumscribed spindle cells in myxoid matrix and multinucleated giant cells; they, characteristically stain positively for S-100, collagen type IV and nerve growth factor receptor; and, are negative for epithelial membrane antigen or markers of histiocytic differentiation. In contrast, the cellular types of neurothekeoma are not encapsulated. The cells are epithelioid with eosinophilic cytoplasm and have rare mitoses; they do not stain S-100, collagen type IV, or nerve growth factor receptor but show reactivity with NK1C3 (CD57) and the panmonocyte marker Ki-M1p. The mixeDual immunoreactivity for neuron specific enolase and S-100 protein in myxoid type support Schwann cell origin, while the absence of S100 protein and positive epithelial membrane antigen in the cellular type suggest perineural cell lineage.Atypical neurothekeomas have been described as resembling either benign or malignant melanocytic growths such as malignant melanoma, Spitz nevus, and cellular blue nevus. In contrast to melanomas that are S-100 positive, cellular neurothekeomas characteristically do not stain with antibody to S-100 protein.Clinically, neurothekeomas are slow-growing asymptomatic lesions. They are commonly dermal, but mucosa and submucosal lesions have been described. It is a 1Neurothekeoma is extremely rare in infancy. Myxoid neurothekeoma may present as dermal lesion in the finger. Reporting such a case will increase awareness about this disease, where it should be included in the differential diagnosis of dermal lesion in infancy and young children. Histopathology with immunostaining helps in diagnosing, classifying  and differentiating  among other soft tissue lesions. Complete surgical excision is curative for these lesions.


Endometrial stromal sarcoma (ESS) formerly classified as low-grade endometrial stromal sarcoma is a rare uterine malignancy with a good prognosis despite a tendency to recur. Primary surgical management for ESS includes total abdominal hysterectomy and bilateral salpingo-oophorectomy. Patients with ESS have long disease-free survival rates when treated with primary surgical therapy, but nearly fifty percent of these patients will recur. We present the case of a patient with recurrent ESS who had an excellent response to combined therapy with megestrol and leuprolide. First reported in 1908, endometrial stromal sarcoma (ESS) is a rare uterine tumor known for its slow growth –4. EndomTypically diagnosed postoperatively on hysterectomy specimens, ESS has an indolent course and recurrent disease can occur up to twenty years after diagnosis. Primary ESS is treated surgically with a total abdominal hysterectomy and bilateral salpingo-oophorectomy and routine lymphadenectomy has not been found to improve overall survival rates , 3, 7, 8A 41-year-old woman gravida 2 para 1 with a body mass index of 25 presented with menorrhagia, pelvic pain, and dysmenorrhea. Her past medical history was unremarkable. Her past surgical history was pertinent for a bilateral tubal ligation in 1991. After examination by her general gynecologist, she was found to have an enlarged uterus. No additional preoperative testing was performed. In November 1996, she underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The frozen section diagnosis revealed a uterine stromal tumor with rare mitotic figures. The final pathology diagnosed an International Federation of Gynecology and Obstetrics (FIGO) stage IA endometrial stromal sarcoma extending to the outer one third of the myometrium . The fal A surveillance computer tomography of the abdomen and pelvis performed in January 1997 showed three 4-5 mm nodular densities in the right lower lobe of the lung. The densities were too small to biopsy and no comparison films were available. The patient was asymptomatic and therefore was managed conservatively. The lung densities remained stable in size until August 1997 when one density was found to have increased to 15 × 14 mm. A lung biopsy of the largest lesion was suspicious, but not diagnostic for a malignancy. The enlarged lung density was thought to clinically represent recurrent disease and the patient was referred to a medical oncologist in her area who recommended cytotoxic chemotherapy. The patient declined chemotherapy and desired a second opinion. After referral to our medical center in November 1997, she was started on a regimen of megestrol 40 mg twice daily with monthly intramuscular injections of leuprolide 7.5 mg. Complete resolution of her lung nodules occurred in response to this regimen by January 1998. She remained on megestrol and a 3.75 mg dose of leuprolide until the date of last followup in June 2006. Now over 10 years after her initial surgery, she remains without evidence of disease.Due to the rarity of ESS, it is difficult to conduct prospective randomized clinical trials to determine the optimal treatment regimen. Treatment has been defined by the experience gained from retrospective case series and case reports. The patient in this case was treated with megestrol and leuprolide based on our clinical experience with this regimen. Our experience with this combination has influenced the use of progestins with GnRH agonists by graduates of our department . Treatment of recurrent ESS is unclear. Chu and colleagues published findings showing that 75% of patients with stage I disease did not recur if treated with adjuvant megestrol compared to 29% of similarly staged patients who did not receive adjuvant megestrol . One casIn conclusion, ESS is a rare uterine malignancy with long disease-free intervals. ESS is rarely diagnosed preoperatively and many patients will develop disease recurrence. We have had success in the long-term management of one patient with recurrent ESS using megestrol and leuprolide and we recommend its use but multicenter prospective trials are needed to determine optimal therapy for this disease entity in the recurrent setting.


Extracranial metastases from brain meningiomas is a rare, but well-documented entity. Metastases occur mostly in the lungs, pleura and liver, but may also affect lymph nodes and bones. We report here on a patient who was treated for an atypical brain meningioma with multiple surgeries and multiple sessions of stereotactic radiosurgery with good control of his brain disease. Thirteen years after diagnosis, he developed bilateral large sacroiliac and abdominal metastases. Meningioma is a common intracranial tumor. Most meningiomas are benign slowly growing tumors that histologically correspond to World Health Organization (WHO) grade I. However, atypical (WHO grade II) and anaplastic (WHO grade III) meningiomas show a more aggressive biological behavior with a high risk of local recurrence and a less favorable prognosis. Extracranial dissemination of meningiomas has been rarely reported. We present here an unusual case of a patient who was treated for an atypical brain meningioma originally diagnosed in 1994 and treated by multiple surgical resections, several courses of stereotactic radiosurgery and radiotherapy, and a course of whole-brain radiotherapy with good control of his cranial disease. His disease remained confined to the brain for thirteen years, after which he developed bilateral large sacroiliac and abdominal metastases.The patient is a 45-year-old man who was admitted for the first time in 1994 at the age of 31 for persistent headache. Work-up revealed a large left tentorial meningioma. He had no history of cranial radiation during childhood or clinical features of neurofibromatosis type I or II. He underwent gross total surgical resection. Pathologic evaluation, which was recently re-reviewed, showed an atypical meningioma with the following features: hypercellularity with sheets of monotonous meningothelial cells displaying a prominent mitotic activity with around 4 mitoses per 10 high power fields. In May of 1997, follow-up brain MRI revealed a recurrence in the vicinity of the original tumor bed. Surgery was performed with gross total tumor removal. Pathologic evaluation again showed meningioma with similar pathologic features. In September 1999, he developed multiple small tumor recurrences in the parietal, occipital and cerebellar lobes for which he underwent stereotactic radiosurgery (SRS) with excellent results on tumor control. In September 2001, the patient developed additional recurrent lesions in multiple sites in the infratentorial, retroclival, left optic nerve sheet, and foramen magnum regions.He was again treated by stereotactic radiosurgery with good tumor control defined as a complete disappearance or progressive decrease in lesion size. In June 2003, he received further SRS treatment to new cerebellar, premedullary and craniocervical junction lesions. In February 2004, he developed numerous lesions outside previously treated sites and was given whole brain radiation therapy (WBRT) of 36 Gy as a palliative therapy because of the multiplicity and the progression of the brain lesions. Surprisingly many of the small lesions disappeared after WBRT and the patient remained asymptomatic until August 2005 when he developed left facial numbness. Brain MR imaging at that time showed a recurrent lesion in the left cerebellopontine (CP) angle, an area that was previously treated by SRS and WBRT. He was given 10 sessions of stereotactic radiotherapy (30 Gy in 10 fractions) and achieved a good partial response but this lesion grew back and caused progressive left facial numbness due to left Vth nerve compression. This was treated by another craniotomy and surgical resection. Histopathologic examination showed meningioma with the same pathologic features and additional elements like the presence of foci of geographic necrosis and a hemangiopericytomatous pattern of vasculature Figure . MitoticIn November 2007 and because of persistent low back pain, the patient underwent MRI of the spine and pelvis. These showed two large masses in the left and right sacroiliac and gluteus muscle regions measuring 11 × 9 cm and 8 × 7 cm, respectively, causing bone destruction of the left sacral wing with extension in the left sacral neural foramina Figure . SurgicaMeningioma is a common intracranial tumor that accounts for 14 to 19% of all primary intracranial neoplasms . DistantSeveral histologic parameters have been identified as indicators of aggressive behavior and predictors of rapid recurrence of meningiomas. These include high cellularity, mitotic rate, nuclear pleomorphism, presence of foci of necrosis, and invasion of adjacent structures -9. OtherAccording to one report, tumors that did metastasize usually had histologic features consistent with a malignant phenotype, such as focal necrosis, brain invasion, cellular pleomorphism, and frequent mitoses . MeningiThe role of chemotherapy is limited in meningioma; there are no or limited proven benefit from any systemic therapy and no clear drug or combination regimen that has given consistent responses.In order of descending frequency, metastases from meningiomas occur in the lungs and pleura, liver, lymph nodes, and bone . Our patIn conclusion, we report here the case of a 45-year-old man who developed bilateral sacroiliac and renal metastases from a recurrent intracranial meningioma of atypical histology 13 years after initial diagnosis.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.Authors' contributions: FG participated in paper editing. MA wrote the manuscript. MK and IAR reviewed the pathology. GH participated in paper editing. All authors read and approved the final manuscript.


We present a case of multiple hydatidosis in an 8-year-old boy that resulted from a ruptured hydatid cyst of the spleen and spread into the peritoneal cavity. We also present a new approach for managing these difficult and high-risk cases. Echinococcus granulosus in humans and many other mammals. The disease is characterized by a worldwide distribution but is endemic mainly around the Mediterranean Sea. There has been recently considerable progress in the diagnosis and management of hydatid disease. Although the radical therapy remains the surgical excision of the cyst, very good results have been reported by the administration of chemotherapy with anthelminthics . Preoperative administration of chemotherapy aims to sterilize the cysts, to soften them, and to reduce intracystic pressure enabling the surgeon to remove the endocyst more easily, whereas postoperatively is used to reduce the rate of recurrence.Hydatidosis is a parasitic disease caused by An 8-year-old boy, Greek, presented in the emergency department with abdominal pain, abdominal distension, diarrheas and fever. A physical examination revealed multiple palpable painless abdominal masses that were mainly confined to the lower abdomen and around the omphalic region. He had a history of acute abdominal pain and anaphylactic reaction with urticaria rash 2 months previously, but this had regressed without medical therapy.Echinococcus granulosus (1/4096). The diagnosis of multiple hydatid disease was established based on imaging findings and positivity for anti-echinococcal antibodies.An ultrasound and computed tomography examination revealed a cyst containing a “floating membrane” in the spleen and numerous unilocular cysts in the peritoneal cavity, measuring from a few millimeters to 3 cm  and 2 FlWe applied chemotherapy  for 6 months, since the spread of the disease into the peritoneal cavity and the multiplicity of lesions were strong contraindications for a radical surgical approach. At around 3 months the cysts in the peritoneal cavity had disappeared and the splenic cyst had reduced in size. However the echinococcal antibody titers, (which were measured monthly), did not fall noticeably throughout the 6-month chemotherapy period.th postoperative day. Six months later the child was well, with a slow decrease in titers and no sign of remaining disease.Our fundamental concerns on exploration were to avoid spreading the disease and to preserve the spleen. A wide trocar was used to open the splenic cyst and suck the contents out. The result was negative since the contents appeared to be gelatinous rather than watery. The splenic cyst cleared and drained externally. The exploration of the abdominal cavity revealed a myriad of cysts, measuring from a few millimetres to 3 cm located only in the great omentum  numerousHydatid cysts compose of three layers: the pericyst, the laminated membrane and the endocyst . The cyst fluid is a transudate of serum that contains proteins and is antigenic . The livImmunodiagnostic tests lack specificity, but they can be helpful in the diagnosis since their sensitivity varies from 60% to 90%. Results of various serologic tests for echinococcus should always be interpreted in conjunction with the patient's medical history, clinical presentation and other findings. The reaction can be present even years after the surgical removal of the cyst, since blood titers decrease only slowly . A serolSurgical excision is still the only curative treatment, but good results have been reported with albendazole administration. Medical treatment is indicated in cysts inaccessible for surgical removal or as a complementary therapy to prevent recurrence . Preoper


Gastric glomus tumors are rare mesenchymal tumors of the gastrointestinal tract. We describe a 72-year-old patient who presented with episodes of melena and was subsequently investigated for a tumor of the antrum of the stomach. Surgical resection revealed a 2 × 2 × 1.7 cm well circumscribed submucosal tumor, extending into the muscularis propria. The histopathologic examination of the specimen demonstrated a glomus tumor of the stomach. We discuss the preoperative investigation, the diagnostic problems and the surgical treatment of the patient with this rare submucosal lesion. Glomus tumors are benign neoplasms of well-differentiated mesenchymal cells. Glomus tumors of the stomach are rare lesions, arising in the intramuscular layer. They typically present as a solitary submucosal nodule in the region of the antrum and pylorus. Preoperative diagnosis of gastric glomus tumors is difficult and requires a multi-faculty medical approach. We present a rare case of a glomus tumor of the stomach along with the investigative procedures and the surgical treatment.Two months ago, a 72-year-old woman presented to her primary care physician with an episode of melena that was suggestive of hemorrhage of the upper gastrointestinal tract. Upon presentation the patient was hemodynamically stable with normal laboratory tests and no evidence of active bleeding in the last 48 hours. Hospitalization was not required and the evaluation was completed in the outpatient department.The patient was subjected to further investigation. Upper gastrointestinal endoscopy revealed mild, diffuse oesophagitis and a small sliding hiatal hernia. At the antrum of the stomach, a 5 cm, well circumscribed submucosal mass with normal overlying mucosa was observed Figure . MultiplThe patient was subsequently referred for surgical consultation. Physical examination revealed a 72-year-old female who was awake and alert, appeared healthy and looked younger than her stated age. Her abdomen was soft, non-distended, without palpable masses. The stool was negative for occult blood. The hemoglobin level was 13.1 g/dL with normal biochemical profile. Tumor markers were within reference ranges. Abdominal radiography showed normal amount and distribution of gas within the bowel. An abdominal computer tomography scan demonstrated a 3 cm localized, prepyloric enhancing mass at the lesser curvature of the stomach Figures . LymphadThe patient was taken to the operative room electively. She was subjected to antrectomy and Roux-en-Y anastomosis. The stomach contained a 2 × 2 × 1.7 cm well circumscribed tumor. Figures . The hisGastric glomus tumor is a benign mesenchymal neoplasm arising from the neuromyoarterial glomus. The glomus apparatus consists of three vascular components: an afferent artery separated from an efferent venole by convoluted channels. Multiple layers of epithelioid cells along with nerve fibers surround these channels . Glomus The first case of gastric glomus tumor was reported in 1951 by Key et al.  and sincGastric glomus tumors present with a variety of symptoms. Epigastric discomfort (intermittent or continuous), hematemesis, melena and occasionally nausea and vomiting can occur. Overt gastrointestinal bleeding has also been reported ,7, in caGlomus tumors are usually solitary. There is only one case report of multiple gastric glomus tumors . Six gloGlomus tumors have to be differentiated from other lesions, such as gastrointestinal stromal tumors (GISTs) and mesenchymal tumors. Preoperative diagnosis of glomus neoplasms is difficult. Glomus tumors grossly appear as red-blue nodules that originate from the muscularis propria ,14. In bEndoscopic biopsies may fail to provide sufficient amounts of material or representative samples of the submucosal lesion and deeper submucosal lesions cannot be reached adequately . Fine neor b) presence of atypical mitotic figure or c) combination of moderate to high nuclear grade and mitotic activity (5 mitoses/50 high-power fields). It should also be mentioned that the classification criteria have been established for superficial or deep soft tissue glomus tumors. However, due to lack of evidence in the current literature, we suggest that the above mentioned criteria should be used by convention for gastric glomus tumors. Only one case of metastatic gastric glomus tumor has been described [Although glomus tumors of the stomach are usually benign, malignant behavior cannot be excluded. Folpe et al.  proposedescribed . The tumHistomorphology of benign gastric glomus tumors is distinctive. Benign glomus tumors consist of small uniform rounded glomus cells that are located in the walls of dilated vessels. The tumor cells have small uniform nuclei, show positive immunoreactivity for smooth muscle actin and are outlined by PAS-positive basement membranes . Glomus Finally, operative intervention should be carefully planned in cases of submucosal gastric masses. All the patients with gastric glomus tumors reported in the literature were operated -7, 9-1616,19. LyPreoperative diagnosis of gastric glomus tumor is difficult. Despite their distinct histological appearance, their clinicopathologic, radiology and upper endoscopy features overlap with more common gastric tumors. The diagnostic gold standard for such lesions is the histological examination and the immunohistohemical markers. A multi-faculty medical approach of the patient optimizes the chances for an accurate preoperative diagnosis and leads to a targeted surgical intervention.Written informed consent was obtained from the patient for the publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journalThe authors declare that they have no competing interests.IV, TT and ND carried out the surgical procedures and contributed to the design of the study. AT gathered the data, drafted the manuscript and critically revised it. AK performed the computed tomography scanning and provided figures for the manuscript along with their interpretation. EK performed the histological analysis of all surgical specimens and provided histological sections as figures of the manuscript. IV revised and finally approved the manuscript for publication.


Myxoid liposarcoma is the second most commonly occurring sub-type of liposarcomas. In contrast to other soft tissue sarcomas, it is known to have a tendency to spread toward extrapulmonary sites, such as soft tissues, retroperitoneum, and the peritoneal surface. Bony spread, however, is not as common.We report an unusual case of diffuse skeletal metastases from myxoid liposarcoma occurring 13 years after treatment of the primary tumour in the left lower limb. The skeletal spread of the disease was demonstrated on MRI only after other imaging modalities  had failed to detect these metastases.MRI is an extremely sensitive and specific screening tool in the detection of skeletal involvement in these types of sarcomas, and therefore, should be a part of the staging process. Liposarcomas are a group of soft tissue malignancies in which the direction of differentiation is toward fatty tissue. The trunk and the lower extremities are the most likely sites of tumour development ,2. LiposA 51-year old Caucasian male presented to his local hospital with a history of an enlarging painful mass in his left thigh in 1992. A needle biopsy was in keeping with the diagnosis of a myxoid liposarcoma, and full staging imaging did not reveal any secondary lesions. A wide local excision was performed and subsequent histopathological examination showed a completely excised high-grade myxoid liposarcoma. Fractions of radiotherapy (60 Gy) were administered postoperatively. In both 1997 and 1999, local recurrences occurred, for which surgical excisions with wide margins were achieved. Surgery was supplemented with radiotherapy on both occasions. In 2004, the tumour disseminated to three distant sites including the liver, chest wall, and neck. The metastases were successfully resected. Histopathological examination revealed similar cellular findings do the primary tumour Figure  showed aet al. [Ishii et al. [Our case demonstrates the possibility of diffuse bone involvement with myxoid liposarcoma, even late in the course of the disease. This uncommon pattern of spread should certainly be taken into account when staging patients and determining their prognosis. It also shows the value of MRI in the detection of these metastases. Other methods for assessing skeletal metastases, including plain radiography, CT scanning and Tc 99m isotope scanning, would appear on the evidence of this, and other cases -9, to beet al.  have shoet al. . We belii et al.  have repi et al. .By reporting this case, we emphasise two important points; diffuse skeletal metastases can occur late in the course of MLS (13 years latency in this case), and MRI appears to be more sensitive than any other imaging modality in detecting bone marrow involvement in this disease. It is therefore essential to consider the value of whole-body MRI in the management of patients with intermediate (myxoid) to high grade liposarcomas. We recommend this be used in the initial staging process, and whenever local recurrence/metastases are suspected.The authors declare that they have no competing interests.SA, YQ, and LB Performed the literature search and drafted the manuscript. LD Contributed to the discussion and the conclusion. PO, IJ, and TWB: Critically reviewed and improved the manuscript.


The distal end of ulna is an extremely uncommon site for primary bone tumors in general and giant cell tumor in particular. Wide resection is usually indicated in such cases and at times it may be necessary to remove of a long segment of the distal ulna. Any ulnar resection proximal to the insertion of pronator quadratus can lead to instability in the form of radio-ulnar convergence and dorsal displacement (winging) of the ulnar stump. This can result in diminution of forearm rotation and weakness with grasp. Stabilization of the ulnar stump after resection for a giant cell tumor was described by Kayias & Drosos. We are adding two more cases to the literature. Both patients had excellent functional outcome and there were no instances of recurrence at three years of follow-up. Giant cell tumour (GCT) of bone is a rare, benign, locally invasive tumour, accounting for approximately 3% to 5% of all primary bone tumours . GCTs ofA 22-year-old Indian male, painter by occupation, presented to us with a painless swelling along the ulnar aspect of his right distal forearm since the last four months. To begin with, it was the size of a peanut but had gradually increased to its present size. There was no history of any other swelling in the body, fever, loss of weight or appetite, or history of similar complaints in the past. The family, occupational, recreational and drug histories were not significant. The general physical and systemic examinations were within normal limits.On examination, there was an oval swelling 6 × 4 cm, occupying the distal third of ulna. The overlying skin was of normal colour and temperature. There was no overlying scar, sinus or prominent veins. The swelling was diffusely tender and homogenously firm in consistency. It was free from the overlying skin but adherent to the underlying bone. The range of motion of wrist was normal and painless. The distal neurovascular status was normal and grasping power equal in both hands.Serum biochemistry studies were within normal limits. Plain radiographs of the right ulna showed an expansile, multiloculated lytic lesion at its lower end with absence of periosteal reaction . Plain cMagnetic Resonance Imaging (MRI) study was planned to delineate the extent of soft tissue involvement, but the patient was very poor and chose not to have the investigation.A clinical diagnosis of GCT was made, which was confirmed post operatively by histopathological examination. The condition, its prognosis and various treatment modalities were discussed at length with the patient.On the basis of clinical and radiographic evaluation, the lesion was graded as Stage 3 (aggressive) as per the Enneking Staging system for benign bone tumors .The tumor resection was extra-periosteal with 3 cm margin of the normal bone proximal to the tumor. This included approximately half (twelve centimetres) of the distal end of ulna, the triangular fibro cartilage complex, the ulnar border of the pronator quadratus and a part of the distal radio-ulnar joint capsule , 3. The Follow-up was carried once every three months for the first two years and six monthly thereafter. Functional evaluation was done using the criteria described by Ferracini . The patA 28-year-old Indian male, shopkeeper by occupation, presented to us with history of pain and swelling on the ulnar aspect of the distal part of left forearm since one year. The swelling was painless and had increased in size gradually. There was no history of fever, loss of weight or appetite, swelling at other sites or history of similar complaints in the past. The family, occupational, recreational and drug histories were not significant. The general physical and systemic examinations were within normal limits.On examination this swelling was comparatively smaller i.e. 3 cm long and 2 cm wide. The overlying skin was normal and free from the swelling. It was firm in consistency and adherent to the ulna. The distal neurovascular status was normal.Plain radiographs revealed a lytic lesion of the distal aspect of ulna with an indistinct medial margin . A proviWe performed a wide resection of the distal 6 cm of the ulna that included 3 cm of the normal bone. ECU stabilization was done in the same fashion as described in Case #1 and the wound was closed over drain.The post operative protocol was similar as described for Case #1. This patient had normal function and muscle strength, no pain or ulnar instability and full range forearm flexion - extension and pronation - supination. He scored 18 out of 18 points on the Ferracini scale.Histo-pathological examination of the resection specimen in both cases was consistent with the diagnosis of giant cell tumor. We achieved tumor free margins in both cases. Radiographic evaluation was done in by measuring the axis between the long axis of the radius and the third metacarpal on lateral view and ulnar subluxation of the carpus on AP view. Both cases had a normal radiographic outcome , 7. TherThe distal end of ulna is a rare site for primary bone tumors, especially giant cell tumour. Tumours at this location can be benign, locally aggressive or malignant. The Enneking staging system for musculoskeletal tumours is useful not only for planning the modality of treatment but also for prognostication. Stage 1 lesions require no treatment, Stage 2 lesions can be managed by extended curettage whereas Stage 3 lesions will need a wide excision [The treatment of giant cell tumors should focus on minimizing the recurrences since it a locally aggressive tumor with a high potential for recurrence. Intra-lesional curettage without adjuvant therapy is associated with a high recurrence rate . It seemThe distal ulna has been traditionally considered as dispensable and resection of the distal ulna for degenerative conditions was first described by Darrach and subsequently modified by Dingman . FunctioMost of the studies on GCT ulna have focussed on wide resection of the distal ulna. In a study on eight patients with various neoplasms of the distal ulna managed by en bloc resection alone, Cooney et al achieved excellent results in 75% of the cases and concluded that reconstruction of the osseous defect is not routinely indicated .On the other hand, many authors have documented failures after wide excision of the distal ulna. This may be attributable to the fact that the ulnar stump has a tendency to displace in a dorsal direction (winging) and also tends to converge towards the radius. This instability can therefore be a cause of persistent pain, weakness with hand grip and limitation of forearm rotation . FurtherTo overcome this problem, many authors have attempted reconstruction or stabilization of the ulnar stump. Gainor et al reported excellent results in two patients treated by a ‘lasso’ tendon graft stabilization of the ulnar stump . In a seMore recently, Roidis et al have described distal ulnar implant arthroplasty as a definitive treatment for a recurrent GCT of the distal ulna. The patient had a good functional and oncological outcome at two years of follow-up .Giant cell tumor of the distal ulna is an extremely rare entity; therefore there are no clear cut guidelines about the preferred modality of treatment. However, most of the authors would agree that a good outcome depends upon two factors: wide resection of the tumor in order to minimize recurrences and stabilization of the ulnar stump in order to achieve a good functional outcome. There is not enough evidence to recommend one ulnar stump stabilization procedure over the other. The authors also feel that the treatment should be individualised, taking into consideration the patient’s age, the length of the ulnar resection and patient’s expectation about postoperative function.


A 23-year-old woman presented to our hospital with nonspecific pain in the abdomen. She underwent radiological investigations, which revealed a solid and cystic mass in the tail end of the pancreas. The mass was diagnosed to be solid pseudopapillary neoplasm of the pancreas on intraoperative scrape cytology. This was further confirmed by histopathology. The cytological diagnosis enabled appropriate surgical treatment to be planned and carried out without undue delay. It is important to distinguish this rare tumor from other pancreatic tumors with similar cytohistologic features because, if diagnosed correctly and managed surgically, this neoplasm is associated with a good prognosis. Solid and cystic papillary epithelial neoplasm of the pancreas (SPENP) was first described by Frantz in 1959 and hence was also called the Gruber-Frantz tumor.1 It formsA 23-year-old woman presented with a two-day history of mid-epigastric and periumbilical pain and also bilious vomiting. The pain was “crampy”, nonshifting and constant. She had periumbilical tenderness on palpation but did not have any rebound pain or guarding. She did not have any associated weight loss, fever, chills, malena, hematemesis, hematochezia or steatorrhoea. Physical examination revealed a deep-seated tender mass in the left upper quadrant.An ultrasound of the abdomen and computed tomography (CT) scan revealed a well-circumscribed, solid mass in the tail of the pancreas measuring 4.6 × 4.7 cm with multiple tiny cysts, which extended up to the splenic hilum. A differential diagnosis of pancreatic endocrine neoplasm, solid pseudopapillary neoplasm and pancreatic carcinoma was considered.A laparotomy was performed and the tumor in the tail of the pancreas along with the spleen was excised. Scrape smears were prepared and stained using rapid hematoxylin and eosin and papanicoloau (PAP) method for cytological evaluation at intra-operative consultation. Highly cellular smears showed numerous delicate branching papillary fronds composed of fibrovascular stalks lined by one to several layers of uniform tumor cells . These tThe resected specimen consisted of the tail of the pancreas, with the tumor and the spleen. The histological features were similar to that of the cytology, consisting of monomorphic cells with ovoid nuclei, fine chromatin, some with nuclear grooving and clear to eosinophilic cytoplasm, arranged in a solid, pseudopapillary, microcystic and trabecular pattern. Amidst the tumor, numerous thin-walled vessels, bands of fibrous tissue, coagulative necrosis and hemorrhage were seen. Also seen were nests of tumor cells extending into the capsule. These features confirmed the cytological diagnosis of solid pseudopapillary tumor of the pancreas. Periodic acid Schiff stain was carried out, which was negative for glycogen, and confirmed our diagnosis.Cystic tumors of the pancreas account for only 6% of exocrine tumors. The various entities in this group occur more frequently in females, but differ in age at presentation. It is important to distinguish SPENP from other pancreatic masses such as mucinous tumors, microcystic adenoma, ductal adenocarcinoma, acinar cell carcinoma and islet cell tumors to enable appropriate surgical treatment because this neoplasm is associated with a good prognosis. A few of227Although the histogenesis of this tumor is uncertain, SPENP is thought to originate from the epithelium of the smallest ductules in the pancreas, which are believed to harbor multipotential cells throughout life. Other au1-antitrypsin, CD56, vimentin and beta-catenin.[Cells are uniform and polygonal with clear to eosinophilic abundant cytoplasm, which is glycogen and mucin negative. Mitotic -catenin. Progeste-catenin.Solid pseudopapillary neoplasm of the pancreas is a distinct clinicopathological entity and is associated with good prognosis. Recognition of the cytological features of this tumor, both preoperatively and intraoperatively, is valuable in planning appropriate surgical management of these patients.


A 20-year-old male patient presented with fungating scrotal mass. Investigations revealed yolk sac tumor with lung metastasis. The patient was treated with systemic chemotherapy. There was complete disappearance of the scrotal mass as well as metastatic disease from the lung. Fungating scrotal mass is a rare presentation of testicular tumor. This rare presentation is reported here. This is second such case in the English literature. The classic presentation for testicular tumors is that of a healthy male in the third or fourth decade of life with a painless, swollen, and hard testis. The presentation can vary with the amount of disease, clinical stage, and the presence of metastasis at the time of referral. Fungating scrotal mass due to testicular tumor is a very rare presentation. Management of such a presentation is discussed here.A 20-year-old male presented in radiotherapy out patient department, with complaints of swelling left testis since 6 months. He was operated 6 months back in a local hospital, where left scrotal orchiectomy was done. The patient did not receive any radiation or chemotherapy after orchiectomy. The swelling recurred with in 2 months and developed in to a fungating mass with foul-smelling discharge.3 cauliflower-like fungating growth arising from the scrotal region covering penis and going up to anterior abdominal wall in its lower part [On local examination, there was a 15 × 12 × 10 cmwer part . It was 3 mass lesion arising from the scrotum. It was irregular in outline and heterogeneous in enhancement. It was reaching up to the level of lower anterior abdominal wall with parietal wall infiltration. The penis was deviated to the right side and was encased by the mass. There were bilateral conglomerate inguinal lymph nodes  of the chest showed multiple ill-defined nodules (1-3.5 cm) in both the lung fields. Some of them were pleural based and peripherally located. Many of the nodules were showing feeding vessel sign suggestive of metastases . The CEC (3-4 cm ). BiopsyThe patient was started on combination chemotherapy with Bleomycin, Etoposite, Cisplatin (BEP) regimen. The mass regressed rapidly due to chemotherapy, locally as well as in the chest leading to complete response. The inguinal nodes also regressed completely. Postchemotherapy tumor markers were with in normal limits. After completion of chemotherapy scrotum was reconstructed by plastic surgeon. The patient is on regular follow-up with no evidence of disease locally as well as in the lungs. On every follow-up, patient was examined clinically, and chest X-ray and USG abdomen were done at completion of treatment and at 4 months. All the tumor markers were normal on follow-up.The incidence of testicular tumors is 2-3/100,000. Scrotal mass is the commonest presentation of testicular tumors. The involvement of the skin is rare, possibly due to tough investing tunica albuginea.[et al.[On literature review, it was found out that this is the second case of fungating scrotal mass due to a testicular tumor being reported in the English literature. First case was also reported from India by Nabi et al. in the yTesticular tumor should be always be kept in the differential diagnosis of fungating scrotal masses, as availability of an effective chemotherapy has improves prognosis of disease even in advanced stages. These tumors respond dramatically to combination chemotherapy with BEP regimen as also seen in our case. These tumors are very sensitive to chemotherapy as well as radiation therapy. Although rare, this case highlights the importance of testicular self-examination and a supportive patient-provider relationship in early diagnosis and improved outcome. All attempts should be made for early diagnosis and treatment of this condition to avoid mutilating surgery. All these tumors respond dramatically to systemic combination chemotherapy. This facilitates less mutilating surgery with no requirement of flap reconstruction and preservation of urethra.