Malignancies are usually preceded by the presence of various paraneoplastic syndromes (PNS), which could be the indirect and/or remote effects of the metabolites produced by neoplastic cells. PNS manifested by oropharyngeal squamous cell carcinomas, which is the most common head and neck malignancy, are highlighted in this review. Knowledge of the clinical spectrum of these syndromes will equip the oral physician for early diagnosis and management of these hidden malignancies, especially of the pharyngeal region. The onset of malignancy may be forewarned by a spectrum of clinical manifestations. Several of these findings are manifested in oropharyngeal squamous cell carcinoma (SCC). As these markers may present before an established cancer, dentist should be equipped with the knowledge to recognize the features in order to facilitate appropriate early intervention and management of the neoplasia which helps the patients lead a better quality life.Paraneoplastic syndromes (PNS) represent a clinical spectrum of manifestations of the indirect and remote effects produced by tumor metabolites or other products and exclude metastasis or any other normal events associated with tumor progression. It is rePNS have been reported in patients belonging to all age groups. The symptoms include manifestations occurring as endocrine, dermatological, hematological, neurological, rheumatological and ocular abnormalities. PNS can precede, follow or be concurrent with a malignancy. It important to differentiate these syndromes from false-PNS; which are the symptoms directly related to the invasion of normal tissue by the tumor or by distant metastases.This article highlights the importance of recognizing PNS caused by oropharyngeal SCC.The exact nature of the paraneoplastic phenomena associated with underlying malignancy is not fully understood. However, it was suggested that neoplastic cells utilizes more than one way to produce components of PNS. Tumor cells can produce hormones, enzymes or fetal proteins, cytokines, stimulate antibody production and metabolize steroids Figure . Any of The symptoms of PNS are dependent on their etiopathogenesis, target organ involved, the quantity and quality of secretion of metabolites and other factors. However, certain manifestations are characteristic of a particular group of tumors.Endocrine symptoms related to PNS usually resemble the more common endocrine disorders.et al. in their review on inappropriate antidiuresis have mentioned about a small group of patients seen with the same inappropriate antidiuretic state but with no demonstrable elevation of ADH. This was considered to be due to increased sensitivity of the vasopressin receptors or due to the secretion of other vasopressive agents immuonologically distinct from ADH.[M Thomson and Adlam DM in their review and case report on ectopic secretion of ADH from the tumor cells associated with oral squamous cell carcinoma (OSCC) have described that the clinical features can range from mild symptoms like fatigue, anorexia and lethargy to severe manifestations like convulsions and coma. This endocrinological manifestation due to the ectopic ADH secretion constitute the syndrome of inappropriate antidiuretic hormone or argentine vasopressin secretion (SIADH). Most patients with SIADH are asymptomatic. SIADH can precede the presentation of cancer by few weeks or months and will recede as the tumor is removed. The persistence of the syndrome despite the treatment for malignancy can be due to continued presence of the tumor. Zerbe etfrom ADH.et al.[et al. in their review on PTH-rP and hypercalcemia have mentioned that the receptor binding and activation domains of PTH and PTH-rP are present with in the first 34 amino acids and both the hormones have identical actions of inducing hypercalcemia by promoting bone resorption and decreasing calcium excretion.[Humoral hypercalcemia of malignancy (HHM) is one of the common PNS manifestations of head and neck SCCs . Iwase Met al. in theiret al. but in mxcretion. Hypercalxcretion.et al. in his review on ectopic ACTH-producing tumors have reported a case of 66-year-old man with laryngeal SCC. One year after the excision, metastasis in the liver was found associated with chemical hyperadrenocorticism. Bioassayable ACTH and MSH were detected in the metastatic tissue.[Imura c tissue.et al. in a 47-year-old man with poorly differentiated OSCC. Patient underwent surgery and radiotherapy but later complained of bilateral breast enlargement and tenderness. Serum β-HCG level was found to be elevated. Later the patient developed axillary node positivity for metastasis. The tumor tissues in the primary and metastatic site were positive for β-HCG. It is reported that such β-HCG-producing tumors usually have poor prognosis.[Paraneoplastic gynecomastia was reported by Scholl rognosis.Ferlito A and Rinaldo A in their review have mentioned that cases of laryngeal SCC has been reported to manifest subacute cerebellar degeneration due to antineural antibodies produced by the tumor cells causing neuronal death. Owing toet al. and Shipley E et al. have reported cases of laryngeal SCC associated with a neurologic PNS called Eaton-Lambart myasthenic syndrome. This PNS usually occurs due to auto antibodies released by tumor cells reducing the release of acetylcholine at the neuromuscular junction causing weakness, myalgias, fatigability especially in the lower extremities and proximal muscles. It is a disorder associated with dysautonomic symptoms that include dryness of mouth and eyes, impotence, diminished sweating and orthostatic symptoms. The symptoms are reported to regress on the removal of the tumor.[Ferroir JP he tumor.Baijens LWJ and Manni JJ in their report of four head and neck cancers associated with PNS has described a case of laryngeal SCC with neck metastasis manifesting paraneoplastic encephalomyelitis. The patient was found to have anti-Hu-antibodies which were directed against the nuclei of central nervous system cells causing inflammation of the grey matter in central nervous system. This disorder consists of five entities determined by the anatomic site of the lesion - they are cerebral encephalitis, brain stem encephalitis, cerebellar encephalitis, subacute myelitis and dorsal root ganglionitis. The brain stem encephalitis typically shows vertigo ataxia, nystagmus, nausea and emesis, when spinal cord is involved symptoms resembling poliomyelitis are seen due to neuronal loss in the anterior and posterior horn of spinal cord. Anti-Hu-positive paraneoplastic encephalomyelitis in association with supraglottic SCC has been reported; another et al.[Laryngeal SCC associated with photophobia was reported by CE Prac et al. in whichet al.Polyarthritis has been reported to present as a PNS. The typical features of that can hint the presence of a hidden malignancy are asymmetric joint disease, explosive onset, predominant involvement of the legs with sparing of the wrists and small joints of the hands with absence of rheumatoid factor, rheumatoid nodules and a family history of rheumatoid arthritis. A sequential relationship should be present between the onset of the articular symptoms and detection of the tumor. The presence of hypertrophic osteoarthropathy or metastatic involvement of synovium or periarticular bone should be excluded. These syet al. in his review on PNS have described about pseudo-stills disease which is an inflammatory arthritis characterized by swelling, tenderness and pain in one or more joint with lymphnode or splenic enlargement. There are few reported cases of laryngeal carcinoma associated with this manifestation.[A case of polymyalgia rheumatica manifested as stiffness and pain in shoulder and hip muscles associated with a hypopharyngeal SCC has been reported in the German literature. Ferlito estation.Hypertrophy osteoarthropathy is a disorder characterized by clubbing of digits, periosteal new bone formation and arthritis. When associated with malignancy they tend to have a rapid onset and progression. They often precede the clinical manifestations of the hidden tumor by months. Deep seated pain in the distal extremities is usual due to periostitis. In patients with malignancies, severe skeletal pain is found before the appearance of clubbing. Few cases of hypopharyngeal SCC are reported to occur with this condition. Makenzie and Scherbel have reported two such cases of laryngeal SCC in their review.Itching is the most frequent cutaneous manifestation in patients with cancer. Herpes zoster and alopecia presenting as part of a PNS are similar to their equivalent benign forms. Immune system depression, which may be observed in most patients with cancer, is often responsible for the reactivation of latent varicella-zoster virus (VZV) in the sensory ganglia.et al. and Miller TR et al. have reported cases of laryngeal SCC associated with this dermatological PNS.[Acanthosis nigricans is a common paraneoplastic manifestation of skin frequently associated with abdominal adenocarcinoma. It is characterized by light or dark areas of hyperpigmentation and warty lesions usually affecting the flexural surfaces. Oral lesions appear as diffuse fine papillary areas most often involving tongue and lips. Oppolzerical PNS.et al. in his report of a case of laryngeal SCC with Bazex syndrome has described the clinical spectrum of this PNS. In the initial stages when tumor is not apparent, the dermatosis is characterized by the appearance of violacious erythema and slight psoriasiform scaling that appears on the hands and feet . Nail dystrophic changes and involvement of aural helixes and nose occur in early stages. Eventually in advanced stages, psoriasiform plaques develop extending centripetally affecting other areas like legs, knees, thighs, arms and trunks. In these advanced stages usually the clinical symptoms of the tumor becomes apparent.[Acrokeratosis Paraneoplastica (Bazex syndrome), is a PNS with dermatological manifestations exclusively associated with malignancies of upper aerodigestive tract including OSCC and metastatic cervical lymph nodes. Miquel JF apparent. The pathapparent.. Most ofapparent.et al. and Renia CC et al. can occur as a PNS of oropharyngeal SCCs. Cohen PR has described the clinical and histopathologic manifestations of Sweets syndrome in his review. Fever is the most frequent symptom but cutaneous manifestations will precede the fever by days to weeks. Skin lesions have a symmetrical distribution, painful and appear as purple/red plaques/nodules or as plaques. These lesions have a transparent vesicle like appearance due to the edema in the upper dermis, some of them can get ulcerated and resemble pyoderma gangrenosum. It is also associated with skin pathergy or skin hypersensitivity which is characterized by the appearance of dermatosis at the site of cutaneous trauma. These include areas of biopsies, IV catheter placement, areas that have received radiation therapy and areas of sunburn. Histopathologically the lesions are characterized by dense neutrophil infiltrate in the upper dermis with edema. The overlying epidermis is normal and changes of primary leukocytoclastic vasculitis are usually absent.[Sweets syndrome (acute febrile neutrophillic dermatosis) as reported by Dawe SA y absent.26Wright JR and Gudelis S from Regional Cancer Centre, Ontario has reported a case of SCC of tonsil associated with digital necrosis as a PNS which was characterized by acral ischemia and multiple necrosis of the finger tips associated with severe pain. Yellow net al.[Paraneoplastic pemphigus is usually associated with autoantibodies from tumor cells against the desmosomal and hemidesmosomal components of epithelium. Occurrence of this disease has been reported in association with tongue SCC by Wong KC and HO KK. Paraneopet al.Spontaneous recurrent or migratory episodes of venous thrombosis, arterial emboli due to non-bacterial thrombotic endocarditis are seen as a PNS in a patient with underlying malignancy. This is due to hypercoagulable materials released from the tumor cells. These thrombotic episodes respond to heparin but not to warfarin. This PNS is known as Trousseaus’s syndrome. They are usually associated with gastric carcinomas but rarely observed in laryngeal carcinomas.et al. reported a case of paraneoplastic polyvasculitis in a 66-year-old patient with underlying hypopharyngeal SCC along with cyanosis, gangrene in the bilateral fingers and toes and pleural effusion. Arteriography revealed several occluded arteries.[Okada M arteries. Leukocytarteries.Symptoms related to erythrocytosis or anemia, thrombocytosis, disseminated intravascular coagulation (DIC), and leukemoid reactions, characterized by the presence of immature WBCs in the bloodstream, are usually accompanied by hypereosinophilia and itching. Thrombocytosis , erythrocytosis and anemia are certain non-specific diseases produced by tumors. Anemia is frequently the presenting symptom of several neoplasms and results from chronic hemorrhages from ulcerated tumors, altered intestinal absorption of vitamins B-6 and B-12, and increased destruction or insufficient production of RBCs. Three types of PNS anemias are described in the literature: (i) Chronic anemia resulting from an anti-erythropoietin factor (ii) microangiopathic hemolytic anemia resulting from DIC, with formation of fibrin filaments in capillary vessels and consequent mechanical hemolysis (iii) Autoimmune hemolytic anemia resulting from anti-RBC antibodies that can be produced by antibodies directed against novel antigens produced by tumors. DIC is typical of epithelial tumors, leukemias and lymphomas .Their occurrence in OSCC, however, is rare and rather non-specific.[PNS are conventionally described as systemic and non-metastatic manifestations associated with a variety of malignancies. They often produce signs and symptoms remote from the site of a malignant tumor, resulting from damage to organs or tissues by mechanisms that are not fully understood but fit into categories as described in PNS caused by oropharyngeal SCC could manifest in a variety of ways described above. They can precede, follow or be concurrent with the diagnosis of SCC, although the diagnosis of PNS is challenging owing to overlap of signs and symptoms. Interestingly, to the best of our knowledge and recent reports, only two (endocrine and dermatologic) of the six main groups of PNS have been reported frequently in association with oral cancer. Humoral hypercalcemia is the most frequent, but it is, in most of the cases, a late manifestation of the oral cancer. Thus it has an important negative prognostic value. Dermatological PNS are less common, but may be a premonitory sign, as they frequently precede the oral tumor. Awareness of this association is important as the first indicator of a malignant tumor or its recurrence.A rapid and correct diagnosis of any PNS may lead to better prognosis. Early recognition of these PNS may lead to clues about the underlying conditions and this may help to avoid diagnostic errors and permit earlier diagnosis and better early intervention. PNS may evolve over weeks to months  and may stabilize, regardless of whether the patient’s symptoms improve or get worse. This article aims to highlight the importance of recognizing PNS associated with oropharyngeal SCCs. Dentists may play an extremely valuable role in the early identification of these conditions through familiarity with these manifestations. Ultimately, timely detection of the cancer-associated syndromes can have an enormous impact on the quality of life and prognosis of this patient population.


Subclavian vein thrombosis is a rare but potentially fatal condition that most often occurs iatrogenically or in the context of malignancy. Here we report the case of an active, healthy 32-year-old woman who presented with subtle findings of arm pain, paresthesias and skin changes of acute onset and was subsequently diagnosed with upper extremity deep vein thrombosis and subclavian stenosis, and was started on a course of oral antithrombotics.A 32-year-old right-handed Caucasian woman presented to her family medicine clinic with left shoulder pain and numbness along her ipsilateral forearm and hand, as well as subtle swelling of the affected limb. Initially diagnosed with medial epicondylitis, she was later diagnosed with subclavian thrombosis caused by Paget–Schröetter syndrome.Presentations such as these are often attributable to soft-tissue injuries that resolve with rest and sometimes physiotherapy. Subclavian thrombosis was a highly unexpected diagnosis in this case; however, family physicians must remain vigilant in considering rare causes of common clinical presentations which could cause patients significant morbidity if left undiagnosed. Nearly one-quarter of adult Canadians attend a physician for musculoskeletal (MSK) disorders each year , making A 32-year-old right (R)-handed Caucasian woman presented to her family medicine clinic with a several-week history of left (L) shoulder pain and numbness along her ipsilateral forearm and fifth finger, exacerbated by elbow extension against resistance. She was initially diagnosed with medial epicondylitis and treated with a non-steroidal anti-inflammatory drug; no differential diagnosis was noted by the original physician but it would have included conditions that can lead to brachial plexopathy, including trauma, compressive tumor, cervical radiculopathy, and spinal stenosis. When she awoke the following morning, her L arm had become swollen and discolored, so she returned to the clinic. On examination, her L arm was subtly swollen  with a similarly subtle purplish discoloration over the dorsal aspect of her distal L forearm  showed stenosis of her R subclavian vein from the thoracic inlet to its origin with her superior vena cava. There was no lung pathology. Unfortunately, characterization of the affected side was not possible with this study and was not repeated. She was referred to vascular surgery. A venogram was ordered there and it demonstrated bilateral obstruction of her subclavian veins, with significant stenosis on the L  side, and complete obstruction on the R  side with stress maneuvers, consistent with bilateral thoracic outlet syndrome and resolved Paget–Schröetter syndrome on the L. She was offered bilateral rib resection and at the time of writing is considering her options.An informal survey of physicians at our family practice teaching clinic revealed only one other case of subclavian thrombosis in the last 16 years, suggesting that in our patient population such presentations are rare. The annual incidence of UEDVT is estimated to be three per 100,000 persons , therefoAlthough surgical decompression and venous angioplasty may be considered for thoracic outlet obstruction ,10,11, tOur patient presented with a 3-week history of shoulder pain and paresthesias limited to the ulnar distribution, followed by an acutely swollen and discolored arm plus paresthesias in the ulnar, radial and median distributions. Initially, our differential diagnosis included cellulitis, lymphedema, and neoplastic compression of the subclavian vein. She had no fever or obvious site of infection to suggest cellulitis; she had had no previous surgery or previous episodes of swelling to suggest lymphedema; and she had no risk factors and displayed no constitutional symptoms to suggest a malignancy. A duplex ultrasound confirmed subclavian thrombosis, and an X-ray and CT ruled out both tumor and a cervical rib as causes for the swelling. CT showed subclavian stenosis on her unaffected side, and venography confirmed bilateral thoracic outlet obstruction with stress maneuvers consistent with Paget–Schröetter syndrome.Although it seemed this primary anatomical anomaly was the probable cause of our patient’s clot, we remained uncertain as to the contribution, if any, of her OCP or her cross-training. She was motivated to continue with exercise, and all of her physicians supported this decision once her INR became therapeutic. Her vascular surgeon supported this approach. She was also considering starting a family soon, and we advised her that she may require anticoagulation with low-weight molecular heparin during future pregnancies. We discontinued her compression sleeve at about 1 month, once her swelling and discoloration had resolved, but vascular surgery recommended she resume using it for at least 12 months, perhaps longer, in order to prevent problems with potential post-phlebitic syndrome.MSK complaints comprise a large majority of presentations to the family physician, and most often they are soft-tissue injuries that resolve with rest and physiotherapy. Occasionally, more sinister causes are uncovered that demand treatment and can result in long-term morbidity. UEDVT is a rare cause of arm pain and sensory changes, but should remain part of the differential in the context of limb swelling and discoloration.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.CT: Computed tomography; DVT: Deep vein thrombosis; INR: International normalized ratio; L: Left; MSK: Musculoskeletal; OCP: Oral contraceptive pill; R: Right; UEDVT: Upper extremity deep vein thrombosis..The authors declare that they have no competing interestsMK saw the patient at her second presentation, made the diagnosis and initiated her treatment. She also performed the literature review and analysis, and contributed to writing the manuscript. VB provided many aspects of the patient’s medical history, continues to see her in follow up and continues to coordinate her investigations. She contributed to writing the manuscript. Both authors read and approved the final manuscript.


Pain was vague and the patient had no sensory complaint at first, but there had been recent exacerbation of pain, since 6 months ago, as sharp radiating pain and paresthesia in 4The first clinical suspicion of physicians was cervical radiculopathy but in magnetic resonance imaging (MRI) there was no disc protrusion/extrusion in cervical and upper thoracic region, and there was a mass lesion at left root of neck .Despite this result the patient was treated as cervical radiculopathy with collars, NSAIDs, and gabapentin for 3 months.With the persistence of pain and paresthesia, she came to our clinic. On the first physical examination, cervical bending was limited by pain, Spurling's maneuver was negative, and pain was aggravated by arm abduction and flexion. There was also mild atrophy of the first interdigital space. By electrodiagnostic examination, increased latency and low amplitude was detected in the left medial antebrachial cutaneous nerve (about 35% of right side). Moreover, in needle electromyography there was increased insertional activity, spontaneous discharges and neurogenic motor units in first dorsal interosseous, extensor indicis, flexor carpi radialis, and left abductor pollicis brevis muscle. The brachioradialis muscle was normal. These findings were compatible with mixed brachial plexus lesion, severe in lower trunk and mild to moderate in medial cord . In sonoThe patient was consulted for surgical excision; open excision through anterior approach (supraclavicular) was done. The prominent bulk of tumor was located in superior mediastinum with extension to neck and serious adhesion to lower part of left brachial plexus especially lower trunk.Pathological assessment revealed totally encapsulated neoplasm composed of spindle cells with no mitosis or necrosis. The final diagnosis was schwannoma. After tumor excision the patient had significant and immediate relief of pain and paresthesia but there was some residual numbness in medial aspect of left forearm and hand after 2 months due to neuropraxia.Schwannomas, neurilemomas, or neurinomas are relatively rare, but benign nerve sheath tumors derive from Schwann cells with low tendency of transformation to malignancy. They are the most common neurogenic tumors and arise from any nerve surrounded by Schwann cells such as cranial, autonomic, or peripheral nerves.4 25% to 45% of extracranial schwannomas occur in the head and neck. A total of 28 consecutive patients were treated between January 2000 and August 2006, for solitary schwannomas in different major nerves of the head and neck. Most affected trunks were cranial nerves in 14 patients (50%), cervical sympathetic chain in 7 (25%), and brachial plexus in 7 (25%). The most common sign was an isolated well-demarcated lesion placement at the lateral aspect of the neck for those tumors arising from vagus, lingual, and sympathetic nerves.2Solitary schwannomas of the head and neck are uncommon tumors arising from any cranial or autonomic nerve.5Extracranial schwannomas usually present insidiously, and thus are often diagnosed incorrectly or after lengthy delays. The most common physical findings associated with brachial plexus tumors are related to local growth. Motor or sensory deficits caused by axonal loss occur more frequently with neurofibromas and malignant nerve sheath tumors. Schwannomas are usually seen as an asymptomatic neck mass, so difficulties in diagnosis are common.Furthermore, the cervical region is a limited space for many vascular and neural structures ; so any mass in this region, accompanied by sensory or motor symptoms or signs, can be a serious alarm for more investigations. In this case the chief complaint of the patient was pain and numbness in upper limb. We could not find any mass in physical examination of the neck, because the main bulk of tumor was in upper mediastinum with extension to brachial plexus.In case of radiating pain and paresthesia in upper limb (as this case) symptoms can be misleading and may suggest cervical radiculopathy. However, careful examination, especially in case of persistence of symptoms and negative imaging results for radiculopathies, is important.


Enteric duplications are rare, but can occur anywhere along the digestive tract. Most of the patients become symptomatic in early childhood and only a few cases of adult patients have been reported in literature. Here we report a unique case of an adenocarcinoma arising in a coincidentally found cystic duplication of the small bowel. The term 'alimentary tract duplication' was characterized by W.E. Ladd to describe those congenital malformations that involve the mesenteric side of the associated alimentary tract and share a common blood supply with the native bowel . EntericA 51-year-old man with no abdominal symptoms was admitted to our hospital with an external computed tomography (CT) scan showing a cystic mass in the mid-abdomen  13.2 μg/l  and CA19-9 55 kU/l .During explorative laparotomy a cystic mass was found in the mesenterium which looked similar to the small bowel but had no connection to the alimentary tract Figure . The surHistological examination revealed a duplication of the small bowel in the mesenterium with nearby physiological architecture. The inner lining mucosa showed indicated villi and crypts and numerous mucous cells. The epithelium showed partly dysplastic areas. At one point it contained a high-grade intraepithelial neoplasia with transition into a poorly differentiated invasive adenocarcinoma infiltrating the muscularis propria Figure .Immunohistochemistry revealed a high expression of CK20 and spot-like CK7. Analogue to the TNM-classification of the small bowel, the tumor was classified as pT2, pN0 (0/25), L0, V0, G2.Postoperative recovery was unremarkable and the patient was discharged six days after surgery. Regular oncologic follow-up in an outpatient setting for one year after surgery showed no hints of tumor recurrence with inconspicuous physical examination and normal tumor marker levels, ultrasound, and CT findings.Enteric duplications (EDs) are rare but can occur anywhere along the digestive tract from the oral cavity to the rectum -7,10. ThEDs usually become symptomatic within the first year of life -7. ReporEDs are most commonly diagnosed when complications like bowel obstruction, perforation, or bleeding occur. Prior to surgery it is difficult to diagnose EDs because of the non-specificity of symptoms and presentation. However, ultrasound, CT scan, and magnetic resonance imaging (MRI) have been useful. Ultrasound can depict the characteristic location adjacent to the bowel and the two-layered wall of EDs ,18-20. BHeterotopic mucosa of gastric or pancreatic origin is a common finding in histological examination of ED . In the Carcinomas arising in duplication cysts are extremely rare complications and only few cases have been reported in literature including carcinoid tumors, squamous cell carcinomas, and common adenocarcinomas -50.Malignant change in small bowel duplications is described most frequently ,22,26-35Histological examination depicted dysplastic areas in the epithelium with an area of a high-grade intraepithelial neoplasia and transition into a poorly differentiated invasive adenocarcinoma. This indicates a tendency to undergo malignant change, which was also reported by Orr and Edwards . MoreoveThe experience of this case and other reports about malignant transformation shows that whenever intestinal duplication is suspected, an immediate operative resection should be performed.Written consent was obtained from the patient for the use and publication of this case report and the accompanying images. A copy of the written consent is available for review from the Editor-in-Chief of this journal.The authors declare that they have no competing interests.GB collected the information, researched the literature, and wrote the article. AK helped with literature research and in preparing the manuscript. BS performed the histological examination and helped prepare the manuscript. RL helped in literature research and edited the final version of the manuscript. All authors read and approved the final version of the manuscript.


Bizarre leiomyoma is a rare leiomyoma variant that requires a precise histopathological evaluation. Especially when diagnosed in a younger woman, this tumor leads to challenging treatment issues involving fertility preservation. Owing to the low incidence of bizarre leiomyoma, there is insufficient evidence to support myomectomy alone as an appropriate management option. Also, the impact of bizarre leiomyoma on fertility is not well known.A 30-year-old Japanese woman who had never given birth was referred to us because of a uterine tumor with an unusual diagnostic image and was treated by a gasless laparoscopic-assisted excision with a wound retractor. Owing to an unclear margin between her uterine tumor and myometrium, a concomitant excision of adjacent myometrial tissue was required to achieve the maximum resection of her tumor. The histopathological diagnosis was bizarre leiomyoma. Seven months later, she conceived spontaneously and her pregnancy course was uneventful. At 37 weeks of gestation, an elective cesarean section was performed. Although a slight omental adhesion was noted at the postexcisional scar, her uterine wall structure was well preserved and a recurrence of bizarre leiomyoma was not noted.A laparoscopic-assisted excision of bizarre leiomyoma is a feasible and minimally invasive conservative measure for a woman who wishes to preserve fertility. Smooth muscle tumors of the uterus encompass a variety of benign and malignant neoplasms . Among uBizarre leiomyoma, also referred to as atypical, pleomorphic, or symplastic leiomyoma, is one of a group of rare leiomyoma variants that require precise histopathological evaluation so that they are not misinterpreted as leiomyosarcomas ,6. AlthoIf fertility preservation is not required, the standard surgical intervention for bizarre leiomyoma that shows a benign clinical course is a simple hysterectomy ,5,6. HowA 30-year-old Japanese woman who had never given birth and who had no disease history consulted her physician for a check-up. Later, she was referred to our department for a uterine tumor with an unusual diagnostic image appearance. During ultrasonography, a uterine tumor that had a maximal diameter of 43 mm and that contained multiple intratumoral cystic lesions was identified (data not shown). On an image obtained by T2-weighted magnetic resonance imaging (MRI), an ovoid heterogeneous tumor in the fundal portion of her uterine corpus was noted Figure , arrow. We informed our patient of the potential risk of hysterectomy if a conservative excision for a uterine tumor with an unusual appearance  was unsuccessful. She declined surgery at that time out of a fear of losing her fertility and chose instead to have her disease condition observed by ultrasonography at periodic check-ups. Since natural conception did not occur and the tumor gradually enlarged to a maximum diameter of 59 mm and the number of cystic structures increased six months later Figure , arrow, A gasless laparoscopic-assisted excision with an Alexis wound retractor  was performed under general anesthesia in accordance with a previous description of the procedure ,9. The lAn en bloc enucleation was extremely difficult because of the fragile and myxomatous nature of the tumor Figure . Thus, bFirst, endometrial defect was closed with Vicryl Rapide sutures (Ethicon Japan). Then, the myometrial defect was reapproximated by two-layered closure with Coated Vicryl sutures (Ethicon Japan) Figure . There wA histopathological examination showed that the excised tumor was composed of numerous large atypical mononucleated or multinucleated cells with eosinophilic cytoplasm, karyorrhectic nuclei, prominent nucleoli, nuclear pseudoinclusions, and coarse chromatin Figure . These bAfter the pathological diagnosis was obtained, our patient was advised to have a regular check-up on purely empirical grounds. During ultrasonography, a remnant of uterine tumor was not evident after the operation, and the postmyomectomy scar healed well. Seven months after conservative surgery, she conceived spontaneously and underwent an uncomplicated pregnancy. At 37 weeks of gestation, an elective cesarean section was performed, and she gave birth to a healthy 3256 g girl with Apgar scores of eight and 10 at one and five minutes, respectively. During cesarean section, a slight omental adhesion to the postexcisional scar was noted. After adhesiolysis, the uterine wall structure was well preserved and a recurrence of bizarre leiomyoma was not noted Figure .Smooth muscle tumors are the most frequent mesenchymal tumors of the uterus . AlthougBizarre leiomyoma is one such rare leiomyoma variant that requires extensive sampling by the pathologist for a differential diagnosis from leiomyosarcoma , especiaExtensive research for a pathological-radiological correlation to enhance the ability to diagnose and manage uterine smooth muscle tumors has been continued ,7,10. TyIn the present case of bizarre leiomyoma, ultrasonography showed an unusual appearance and multiple low-echoic cystic structures within the tumor. A signal intensity that was mostly higher inside the tumor than in the surrounding myometrium on T2-weighted MRI images was another characteristic diagnostic image feature of the present case. When a surgical strategy is planned, a combination of these diagnostic image findings is the potentially important clue to narrow the preoperative diagnosis of this unusual variant of leiomyoma in a woman who wishes to preserve fertility.If fertility preservation is not a major concern, a simple hysterectomy performed either initially or secondarily after the diagnosis of bizarre leiomyoma in a specimen obtained from myomectomy is a definitive curative treatment for bizarre leiomyoma that shows a benign clinical course ,5,6. HowIn the present case, it was assumed that bizarre leiomyoma deforming the uterine cavity was the significant cause of infertility since spontaneous conception occurred early after the operation. Although the present report suggests that conservative excision of bizarre leiomyoma could be an effective measure for a woman who wishes to preserve fertility, difficult identification of the dissecting plane because of an unclear border between the tumor and the adjacent myometrium during myomectomy should be cautioned before considering a conservative surgical intervention. Insufficient resection may cause early tumor recurrence; however, over-resection of adjacent myometrium is associated with a potentially dangerous defect of the uterine wall and may lead to uterine rupture during a subsequent pregnancy.Reports by Sesti and colleagues -13 indicLaparoscopic-assisted excision of bizarre leiomyoma is a useful and minimally invasive conservative measure for a woman who wishes to preserve fertility, although close follow-up for an extended period of time by periodic ultrasonography for a possible recurrence is warranted. Additional conservatively managed cases with bizarre leiomyoma are required to confirm the safety and reliability of the enucleation procedure and to enable counseling of future patients who wish to preserve fertility.MRI: magnetic resonance imaging.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.AT collected the patient data and was involved in drafting the manuscript. SI, MM, and HN were involved in patient care. All authors read and approved the final manuscript.


We report the case of a 12-year-old girl, who consulted us with one-year history of an 8 mm nose lesion that was painless and firm upon palpation. The lesion was resected conservatively. Immunohistochemistry was in favor of a primitive neuroectodermal tumor (PNET)/Ewing's sarcoma lesion, excluding epithelial, lymphoid, and other tumors. After a second resection, our patient was referred to chemotherapy and has already undergone 9 cycles out of 14. The patient is to date with no evidence of persistent or recurrent disease. To our knowledge, this is the first description of a PNET arising in the nose. Primitive neuroectodermal tumors (PNETs) are a large family of tumors of neuroectodermal origin that belong to Ewing's sarcoma family of tumors –4. Head The authors report here the case of a 12-year-old girl that presented with tip of nose lesion a year ago. This lesion was asymptomatic and barely increased in size in the first 3 months. She presented to our clinic in September 2011 with an 8 mm lesion that was painless and firm upon palpation . This leleukocyte common antigen (LCA), cytokeratin cocktail (CKC), synaptophysin, chromogranin, S-100, CD99, myogenic differentiation 1 (MyoD1), glial fibrillary acid protein (GFAP), neuron-specific enolase, and Ki-67 according to a standard immunohistochemical protocol. Tissue from the tumor was processed routinely for light microscopy. Immunohistochemistry was performed in paraffin-embedded tissue using commercially available antibodies against Histology revealed a neoplastic malignant polypoid proliferation, located in the lamina propria under an intact stratified squamous epithelium, composed of sheets of small, round to oval blue cells, arranged in lobules and separated by fibrous septa. Cells were characterized by scant, ill-defined cytoplasm and well-defined nuclei with coarse chromatin pattern. There was no rosettes formation. No areas of necrosis were noted, neither signs of differentiation toward heterologous elements. CD 99, S-100, and neuron-specific enolase and negativity for the other markers, with a proliferation index superior to 15% in favor of a PNET/Ewing's sarcoma lesion, excluding epithelial, lymphoid, and other tumors  is usually present as well as other neural differentiation markers such as neuron-specific enolase, S-100, synaptophysin, and chromogranin . AlthougThere are no characteristic radiologic presentations but peripheral PNET (pPNET) presents as a heterogeneous mass on CT scan, isointense to muscle on T1-weighted MRI but with homogeneous enhancement with gadolinium and nonhomogeneous hyperintensity on T2-weighted MRI –11. ThesThe most difficult task is to differentiate pPNET from Ewing's sarcoma, and this diagnosis is made on the basis of neural differentiation since both entities represent a spectrum of the same disease , 8.Combination treatment modalities are used in this type of tumor, most frequently surgery followed by radiation for local control and chemotherapy. Inoperable tumors are treated with concomitant chemoradiation protocols –5. RecomPrognosis is usually poor with less than 50% of disease-free survival at three years and 30%–45% at 5 years , 4, 5. P


A 46-year-old previously fit lady was admitted with acute pancreatitis. She had no history of gallstones. She was not on any medications and consumed minimal amounts of alcohol. On subsequent investigations as to the causative factor, she was found at ultrasound to have an air-fluid filled cystic structure posterior to the head of pancreas which was compressing the common bile duct. Further magnetic resonance imaging and computer tomography scans showed that this cystic lesion was located around the ampulla of Vater. A diagnosis of a perivaterian abscess was made. At endoscopy, a large contained abscess was seen which was successfully drained. She made a full and uneventful recovery. We present a case of acute pancreatitis caused by a duodenal diverticular abscess occluding her ampulla of Vater. This case report is the first documented case in the literature of acute pancreatitis caused by a perivaterian duodenal diverticular abscess.A thirty-eight-year-old female presented to our emergency department with one-day history of acute onset epigastric pain. It was constant and sharp in nature and associated with several episodes of vomiting. There was no history of fevers, rigors, dysuria, or change in bowel habit. She gave a two-week history of preceding mild abdominal pain, particularly after eating. She was previously fit and well with no history of gallstones, took no regular medications, and had no significant past medical history. She had no known drug allergies and was on no regular medication. She was a nonsmoker and consumed around four units of alcohol per week.On inspection, she looked unwell. She was apyrexial and tachycardic. On palpation of her abdomen, she had marked epigastric tenderness and guarding. Her blood tests revealed a raised white cell count (16.5), bilirubin (26), ALT (165), AST (222), LDH (494) and amylase level of 2245. She was diagnosed with acute pancreatitis, scoring 2 on the Glasgow severity score.An abdominal ultrasound demonstrated a dilated common bile duct (11 mm) with a distended gallbladder with no gallstones or other pathology . A MRCP performed the following day confirmed a 12.4 mm dilated bile duct with dilation of the intrahepatic biliary tract. In addition, it revealed a “curious” cystic lesion, with an air-fluid level, lying posterior to the head of the pancreas at the level of the distal common bile and pancreatic duct, which appeared to be causing some extrinsic compression . She began to improve clinically and haematologically (reduced leukocytosis) with oral antibiotics. However her bilirubin continued to rise, peaking at 58. She underwent a CT scan of her abdomen on her fourth day of admission. This showed a cystic or necrotic mass behind the head of the pancreas, representing an inflammatory lesion, phlegmon, or duodenal diverticular abscess .An ERCP on her sixth day postadmission revealed a 10 cm duodenal diverticular abscess draining pus and bile, at the level of the major papilla . It appeThis is the first documented report of a perivaterian diverticular abscess causing acute pancreatitis due to compression of the ampulla of Vater. Duodenal diverticula occurs with a frequency of between 5% and 25% . The larRecognised complications of these types of diverticulae include mechanical obstruction and perforation leading to peritonitis, requiring urgent surgical intervention. Ulceration giving rise to upper gastrointestinal bleeding has also been reported and could be fatal if the erosion involves the aorta or a mesenteric vessel , 4.Radiological diagnosis of these abscesses can be difficult. Since the commonest site of formation is at the second part of the duodenum, a large fluid-filled cystic lesion could easily be diagnosed as a neoplasm of the pancreas arising from the head of the pancreas, which is the common site of pancreatic neoplasm formation. Computer tomography and magnetic resonance imaging scans are useful to distinguish between these two widely varying diagnoses by demonstrating characteristic air-fluid levels within these lesions , 6. UppeIt has been shown that there is an association between periampullary diverticula, which can lead to abscess formation, and biliary duct stones. However a large study showed that there is no association between periampullary diverticula and pancreatitis . Thus inOur presented patient was successfully treated with endoscopic drainage of this abscess and made a full uncomplicated recovery. She was seen four months postoperatively in clinic with no reported problems. She is now being routinely followed up at our local tertiary hepatobiliary centre. Endoscopic relief for similar cases has been reported successfully in several other cases , 9 and iThis is the first documented case of a perivaterian duodenal abscess causing compression of the common bile and pancreatic duct leading to pancreatitis. Duodenal diverticula are more common than previously thought, but case reports are scarce as the majority are silent and cause no significant clinical manifestations. Care must be taken to diagnose the condition correctly by using appropriate imaging modalities. Successful endoscopic treatment is possible and should be attempted in appropriate patients.


Primary adenocarcinoma of the rete testis is an extremely rare extratesticular neoplasm. Due to its low occurrence and the scarcity of data, sonographic characteristics of adenocarcinoma of the rete testis are still poorly defined. A 46-year-old male complained of swelling and pain in the right side of the scrotum. No associated symptoms were observed. Anti-tuberculosis chemotherapy yielded no response. Postoperative pathology revealed a diagnosis of poorly-differentiated adenocarcinoma of the rete testis. Using the ultrasonography features observed, combined with a review of current literature, the diagnosis and differential diagnosis of this neoplasm are presented. Adenocarcinoma of the rete testis is a rare, highly aggressive tumor originating from the nonspermatogenic epithelium of the intratesticular excretory ducts. Only ~60 cases have been reported in the literature to date. Rete testis adenocarcinoma occurs most frequently in elderly males and is usually associated with a poor prognosis . The majA 46-year-old male was admitted with complaints of swelling and pain in the right side of the scrotum for a 1-year period. No associated symptoms were observed. Anti-tuberculosis chemotherapy was performed in Sichuan Provincial People’s Hospital , which yielded no response. Physical examination revealed a swelling of the right scrotum and hard nodules at the head of the right epididymis exhibiting severe tenderness. Written informed consent was obtained from the patient.Ultrasonography of the scrotum revealed a 1.0×2.1-cm hypoechoic nodule at the right epididymis with a poorly-defined border. In addition, a relatively abundant blood flow was detected by color Doppler ultrasound and a weak echo was recorded in the right side of the tunica vaginalis area . A 5.0×1The levels of lactate dehydrogenase (LDH), α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-HCG) were normal in the serum. Chest radiographs, abdominal ultrasonography and computed tomography (CT) of the abdomen revealed no remarkable results. Considering the aforementioned results, a diagnosis of primary testicular tumor was proposed.Based on this provisional diagnosis, scrotal incision was performed for testicular exploration. This revealed blood-mixed fluid in the scrotal skin and an enlarged right epididymis with multiple yellow-grey, rough nodules, forming adhesions with the scrotal skin. Intraoperative frozen sections revealed adenocarcinoma in the specimen and the scrotal incision was extended and a high right inguinal orchidectomy was performed.Light microscopic examination revealed an absence of normal testicular microstructure, with nests of cells separated by fibrovascular stroma. In addition, a transition from normal to tumorous epithelium was detected. A small portion of lumens of the rete testis were packed with cuboidal tumorous cells. Cells exhibited heterotypic hyperplasia and were partially arranged in a glandular pattern, with hyperchromatic nuclei, an increase in the nuclear/cytoplasmic ratio and a visible nucleolus . No invoTaking into account that adenocarcinoma of the rete testis is highly resistant to adjuvant radiotherapy and any known chemotherapeutic regimens ,6, no fuPrimary adenocarcinoma of the rete testis is resistant to adjuvant therapy and is associated with a poor prognosis. As many as 40% of patients succumb to this condition within one year of diagnosis. Survival rates for 3- and 5-years are 49 and 13%, respectively –9. ThereUltrasonography provides information regarding composition of the lesion which may facilitate diagnosis and differentiation from other pathological tumor types. Adenocarcinoma of the rete testis is typically located in the region of the epididymis or testicular hilum, rather than the intratesticular region, as reported in the majority of the current literature ,8,13,14.In a number of cases, other imaging methods, including CT, magnetic resonance imaging and nuclear medicine, may be necessary to complete the imaging work-up of patients with testicular tumors. However, pathological examination is still the gold standard for diagnosis confirmation.In conclusion, adenocarcinoma of the rete testis is an extremely rare tumor type with a poor prognosis. Sonography is the most promising tool for early diagnosis and increased case examples providing sonographic tumor observations must be presented to achieve an improved rate of diagnosis.


Fibrous dysplasia (FD) is categorized as either monostotic or polyostotic and may occur as a component of McCune-Albright syndrome (MAS). Imaging findings can mimic neoplastic diseases. We present a case of MAS initially suspected to have neoplastic disease. A 9-year-old girl was admitted to pediatric emergency with ataxia. Upon hospitalization, an extradural mass was seen on cranial magnetic resonance imaging (MRI) and the bone survey showed lytic lesions in the long bones. The patient was referred to the pediatric oncology department with a presumptive diagnosis of Langerhans cell histiocytosis or metastatic tumor. Further investigations demonstrated that the patient had MAS and coexisting postinfectious cerebellitis. The findings in this patient demonstrate that the radiographic findings and the clinical presentation of FD and MAS may be similar to those of malignant diseases. Conflict of interest:None declared. Fibrous dysplasia (FD) is a common benign disorder of the bone in which fibro-osseous tissue replaces the bony spongiosa. It comprises around 7-10% of all benign bone tumors. 70-80% of FD cases are monostotic and 20-30% are polyostotic . ComputeWe report the case of a patient who had multifocal bone lesions and ataxia and was referred to our clinic with a presumptive diagnosis of malignancy. The patient was subsequently diagnosed as MAS and postinfectious cerebellitis. th month of pregnancy without an identified pathology. The patient’s history did not reveal any recent immunization, upper respiratory tract infection, diarrhea or drug use. On inspection, the patient appeared to be in a state of exhaustion. Her blood pressure was 110/70 mm Hg, respiratory rate 24/minute and pulse rate 80/minute. Her height was 135 cm  and weight was 27 kg (25-50th perc.). Her body mass index (BMI) was 14.8. Physical examination findings were within normal limits except for ataxia. There were two irregularly bordered café au lait spots - one on the right cheek and the other in the presacral area. Breast development and pubic hair growth both corresponded to Tanner stage 4. A 9-year-old girl was admitted to the pediatric emergency department because of a 2-day-history of unsteady gait, vomiting, and dizziness. She was the third child of four pregnancies to a non-consanguineous couple. Her prenatal/natal history was unremarkable and her two siblings were healthy. One sibling had died in utero at the 8Initial laboratory findings were within normal ranges. A presumptive diagnosis of acute postinfectious cerebellitis was made. Parenteral hydration and acyclovir therapy were initiated. Otolaryngology consultation was sought. An inner ear problem as cause of ataxia was considered, but could not be proven by audiological examination. Cranial MR was done to delineate central nervous pathologies and revealed that there were fusiform expansions reaching 1.5 cm in thickness in almost all skull bones with a special predominance in the occipital and right parietal bones. Moderate diffuse contrast enhancement was also noted in these bones. Brain parenchyma was normal in terms of white and grey matter. The radiological differential diagnosis included Langerhans cell histiocytosis, primary bone lymphoma and neuroblastoma metastasis. CT scans supported the MRI findings except for a ground glass appearance indicative of FD . 99mTc MDP scintigraphy demonstrated extensive multiple osteoblastic uptake in accordance with either histiocytosis or FD. The bone survey findings were as follows: loss of the distinction between cortex and medulla at the cranium, fingers and toes, as well as the humeri, radii, and ulnae, in addition to disrupted trabecular structure and presence of radiolucent areas. 6/12 years before the current situation. Precocious puberty, café au lait spots, and polyostotic FD in radiological differential diagnosis pointed to MAS. However, due to a suspicion of malignancy and presence of coexisting ataxia, a bone marrow biopsy was performed to exclude malignant infiltration. The biopsy represented both cortical and medullary areas. There were no signs of neoplasia, but the trabeculae were thin and mimicked Chinese letters, lacking osteoblastic rimming and were surrounded by a moderately cellular fibroblastic proliferation. The findings were consistent with FD. The lytic and expansive bone lesions raised the suspicion of malignancy, and the patient was referred to the department of pediatric oncology. Re-evaluation of the patient's history revealed that the breast development and pubic hair growth had started 28/12 years.The patient was therefore referred to the department of pediatric endocrinology. The patient’s bone age was 11 years. Thyrotropin (TSH) was found to be slightly elevated (5.07 mU/mL). Serum alkaline phosphatase level was 738 U/L, Ca 10 mg/dL, and phosphorus 5 mg/dL. Her DEXA results revealed a Z-score (lumbar spine) of -1.9. The patient was diagnosed as a case of MAS. Her symptoms disappeared after 10 days of acyclovir treatment and supportive measures. She was discharged to be followed up jointly by the orthopedics and pediatric endocrinology departments. The patient had menarche at age of 9FD is a developmental anomaly of the bone formation in which the normal bone and marrow are replaced by fibrous tissue and small, woven spicules of bone. FD may exist in a monostotic or polyostotic form. MAS is characterized by a combination of FD, cutaneous pigmentation, and endocrine abnormalities . The mutThe diagnosis of MAS is usually established on clinical grounds. The medical history including extraskeletal manifestations should be taken into consideration. The initial evaluation of our patient failed to reveal her precocious puberty and therefore MAS was not among the differential diagnoses considered for the multifocal skeletal involvement. The diagnosis became evident when the concomitant presence of polyostotic FD, café au lait spots, and precocious puberty was evaluated. Unfortunately, [i]GNAS[/i]mutation testing was not available in our facility. The detection of a bone mass with/without limp or fracture poses a diagnostic dilemma for the general pediatrician or orthopedic surgeon in deciding whether the mass is cancerous or indicates FD. A radiograph is usually the first imaging modality to be performed. The plain film reveals a lytic, ground glass lesion in FD. In case of doubt about the diagnosis, the CT is helpful in demonstrating the medullary-based, fibro-osseous nature of FD . After tAfter diagnosis, MAS patients are referred to pediatric endocrinology clinics. However, the clinical dilemma may still persist in MAS cases presenting with mass lesions. [i]GNAS[/i] mutations responsible for MAS lead to adenylyl cyclase-mediated increase in cAMP which initiates uncontrolled cell proliferation . Thus, FSome of the MAS cases may present with atypical initial features such as ovarian masses and testicular enlargement. In an otherwise healthy child who presents with a pelvic/testicular mass, the distinction between malignant and benign lesions can be challenging. Such patients may undergo unnecessary diagnostic or therapeutic interventions. Nabhan et al  reportedConflict of InterestThe findings in this patient demonstrate that the radiographic findings and the clinical presentation of FD and MAS may be similar to those of malignant diseases.


In more than 98% of cases, acromegaly is due to a GH-secreting pituitary adenoma. The term “ectopic acromegaly” includes neuroendocrine tumors secreting GH releasing hormone (GHRH), usually located in the lungs, thymus and endocrine pancreas. Considerably less frequent are cases of ectopic acromegaly due to GH-secreting tumors located out of the pituitary fossa; except for one isolated case of a well-documented GH-secreting lymphoma, the majority of these lesions are located in the sphenoid sinus.We present the case of a 45 year old woman with acromegaly whose MRI showed an empty sella without evidence of a pituitary adenoma but revealed a large mass within the sphenoid sinus. She underwent transsphenoidal surgery and the excised sphenoid sinus mass, proved to be a GH-secreting adenoma; the sellar floor was intact and no other lesions were found in the pituitary fossa. She required postoperative treatment with somatostatin analogs and cabergoline for clinical and biochemical control.This case highlights the importance of carefully evaluating the structures surrounding the sellar area when a pituitary adenoma is not found with currently available imaging techniques. The finding of an intact sellar floor and duramater lead us to conclude that the patient’s tumor originated de novo from embryological pituitary remnants. Upon a careful review of the literature and a critical evaluation of our case we found neither clinical nor biochemical features that would distinguish an ectopic from the more common eutopically located somatotrophinoma. Acromegaly results from an excessive GH secretion by a pituitary adenoma in over 98% of the cases . It is a111In-labeled octreotide scintigraphy [Ectopic acromegaly due to a GHRH neuroendocrine tumor (NET) should be suspected when a patient presents with all the clinical and biochemical features of the disease but without evidence of a pituitary adenoma on MRI ,5,6. In tigraphy ,5,6. Thetigraphy . In thestigraphy .We here in report the case of a woman with acromegaly without a pituitary lesion in whom GH-secreting adenomatous tissue in the sphenoid sinus proved to be the source of her hypersomatotrophism.A 45-year-old woman presented with a 5-year history of menstrual abnormalities, knee and elbow arthralgias, hands and feet enlargement, coarsening of facial features, fatigue, dental spacing and symptoms of obstructive sleep apnea (snoring and day-time sleepiness). On physical exam her blood pressure was 180/100 mmHg, pulse 80 and regular, weight 65 Kg, height 1.55 m. She had evident acral enlargement with prominence of supracilliary arches and nose bridge, prognathism and dental spacing. Numerous pigmented skin tags were visible in her anterior chest wall and there was slight acanthosis nigricans in her neck and axillary regions. Her thyroid was palpable and somewhat nodular but without distinct lesions. The diagnosis of acromegaly was confirmed by an age adjusted IGF-1 level 4.8 times the upper limit of normal (ULN) and a basal and post glucose GH concentrations of 7.7 ng/mL and 2.5 ng/mL, respectively ; the excised adenomatous tissue was analyzed both by HE and immunohistochemistry for pituitary hormones, and proved to be a GH-secreting tumor. The surgical findings support the hypothesis that our patient’s adenoma originated from pituitary embryological remnants located in the sphenoid sinus. This is probably the first case of ectopic acromegaly due to a sphenoid sinus adenoma to be treated successfully with a combination of a somatostatin analog and a dopamine agonist.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 of this journal.GH: Growth hormone; IGF-1: Insulin-like growth factor type 1; LH: Luteinizing hormone; FSH: Follicle stimulating hormone; PRL: Prolactin; TSH: Thyroid stimulating hormone; FT4: Free thyroxine; GHRH: Growth hormone releasing hormone; MRI: Magnetic resonance imaging; CT: Computed tomography; ULN: Upper limit of normal; CBG: Cabrgoline.The authors have no conflict of interest to declare.CR and MM drafted the manuscript. CR, LH, JMF and ALE collected all medical reports of the patient. ALE and MM were the neuroendocrinologists in charge of the case and GG was the neurosurgeon who performed the transsphenoidal resection of the tumor, contributing the technical aspects of the procedure and its findings. MM coordinated the group. All authors read and approved the final manuscript.Claudia Ramírez, MD, MSc: Endocrinologist, Associate Professor of Medicine Universidad Nacional Autónoma de México Faculty of Medicine. Staff investigator, Experimental Endocrinology Unit, Hospital de Especialidades, CMN S.XXI, IMSS, México City. Research interests: Pituitary adenomas, Neuroendocrine tumors.Laura-Cristina Hernández-Ramirez, MD: Endocrinologist, currently completing her PhD in molecular endocrinology at the Centre for Endocrinology, William Harvey Research Institute, Barts and The University of London School of Medicine.Ana Laura Espinosa de los Monteros, MD: Associate Professor of Medicine Universidad Nacional Autónoma de México Faculty of Medicine. Senior attending endocrinologist, Endocrinology Service, Hospital de Especialidades, CMN S.XXI, IMSS, México City. Member of the National System of Investogators. Research interests: Pituitary adenomas, acromegaly and Cushing’s disease.Juan Manuel Franco: Sixth year medical student, Universidad Nacional Autónoma de México Faculty of Medicine. Currently completing a 12-month research fellowship at the Experimental Endocrinology Unit, Hospital de Especialidades, CMN S.XXI, IMSS, México City.Gerardo Guinto, MD: Professor of Neurological Surgery Universidad Nacional Autónoma de México Faculty of Medicine, Head of the Neurosurgery Service, Hospital de Especialidades, CMN S.XXI, IMSS, México City. Member of the National System of Investigators. Reviewer for Journal of Neurosurgery, Neurosurgery  and World Neurosurgery. Research interests: Skull base and pituitary surgery.Moises Mercado, MD, FRCP(C): Professor of Medicine Universidad Nacional Autónoma de México Faculty of Medicine. Director Experimental Endocrinology Unit, Senior Consultant, Endocrinology Service, Hospital de Especialidades, CMN S.XXI, IMSS, México City and ABC Medical Center Neurological Unit. Member of the National System of Investigators, Editor-in-chief Revista de Endocrinologia y Nutrición. Reviewer for J Clin Endocrinology and Metab , Endocrinology, Pituitary, Clinical Endocrinology, Endocr Pract, Eur J Endocrinol, Arch Med Res. Research interests: Clinical, therapeutic and pathophysiological aspects of pituitary tumors, particularly acromegaly, nonfunctioning tumors and Cushing’s disease.Justification: Ectopic Acromegaly due to GH-secreting tumors located out of the pituitary fossa is an extremely rare condition. We present a thoroughly evaluated case and performed an in-depth review of the literature in an attempt to find distinctive clinical or biochemical features that would aid in the diagnosis and treatment of these patients.Potential reviewers: Oscar Bruno, Universidad de Buenos Aires (bodomingo@intramed.net), Ashley Grossman, Oxford University (ashley.grossman@ocdem.ox.ac.uk), Philip Chanson, Hopital Bicetre, Universite de Paris (philippe.chanson@bct.aphp.fr)


A 23-year-old man presented with a mass in the pineal region and obstructive hydrocephalus. A neuroendoscopic biopsy for the lesion, ventriculoperitoneal (VP) shunting, and focal irradiation were conducted as initial treatment. Histological diagnosis of the biopsy specimen was germinoma. He underwent further irradiation and two tumor resections. Histological diagnosis was mature teratoma without a germinomatous component. After serial treatments, the intracranial lesion was controlled. However, 14 months after presentation, extraneural lesions were confirmed in the posterior mediastinum and retroperitoneal space. The biopsy specimen of the retroperitoneal space lesion was histologically diagnosed as germinoma. Although chemotherapy with cisplatin and etoposide was undertaken, extraneural lesions ware uncontrollable and he died. At autopsy, extraneural lesions were confirmed in the posterior mediastinum, retroperitoneal space, and right lung. Histological diagnosis of extraneural lesions was germinoma. This case was considered to be a pineal mixed germ cell tumor mainly consisting of germinoma and mature teratoma, which caused hematogenous metastasis of the germinoma component. Systemic chemotherapy and irradiation for primary lesions as an initial treatment is important to cure the primary lesion and prevent extraneural metastasis. Extraneural metastases of primary brain tumors are rarely reported. Extraneural metastasis of primary brain tumor is thought to occur in situations such as direct local invasion, hematogeneous or lymphatic spread, and seeding via the cerebrospinal fluid pathways. The most common such brain tumor is glioblastoma, followed by medulloblastoma and ependymoma. There are few reports of patients with hematogeneous metastasis associated with intracranial germ cell tumors –3. Most A 23-year-old man was transferred to our department because of progressive consciousness disturbance. He was drowsy on admission. A CT scan revealed a well-demarcated and heterogenous mass with calcification in the pineal region. The mass was approximately 40 mm in diameter, and extended into the lateral ventricles, causing obstructive hydrocephalus Fig. . Based o2 and etoposide 50 mg/m2 was undertaken, but discontinued because of allergy and poor general condition. Although the lesions transiently reduced in size during treatment, they enlarged rapidly when treatment was discontinued, coinciding with a sudden deterioration of the patient’s general condition. Eighteen months after presentation, the patient died due to sudden shock. At autopsy, the cause of death was stated as hemorrhagic shock due to an intratumoral hemorrhage in the retroperitoneal space lesion.Fourteen months after presentation, chest and abdominal CT scans for examination of unknown fever revealed masses in the posterior mediastinum and retroperitoneal space Fig. . Serum AThe biopsy specimen of the intracranial lesion showed the characteristic two-cell pattern of a germinoma with large clear cells and small lymphoid elements Fig. a. ImmunoAt autopsy, macroscopic observation showed that extraneural lesions were found in the posterior mediastinum, retroperitoneal space, and right lung Fig. a.Fig. 5MThe posterior mediastinal lesion was 8 cm × 6 cm in size, and located posterior to the tracheal bifurcation. The mass contained whitish necrotic tissue. The retroperitoneal space lesion was 17 cm × 15 cm in size and was associated with intratumoral hemorrhage. The mass encased retroperitoneal structures such as the aorta, left adrenal gland, and duodenum. The pulmonary lesion was in the right middle lobe. Histologically, extraneural lesions were diagnosed as germinoma (Fig. Germ cell tumors occasionally disseminate to the ventricular system and spinal subarachnoid spaces . HoweverSugiyama et al.  investigAlthough our patient underwent VP shunting, metastasis via shunt tube is deniable because the metastatic lesions arose in the retroperitoneal space, and there were no lesions in the abdominal cavity at autopsy. In some patients with primary intracranial germ cell tumors, metachronous lesions in the midline structures have been reported. According to the literature, metachronous lesions developed in the different site of the brain, mediastinum, or testis 8 months to 16 years after complete remission of the primary intracranial lesion –9. As faHematogenous metastasis from germinoma is rare, only 13 cases have been reported –21. ThesGerminomas are often cured with radiation. In our case, the germinomatous component in the primary region disappeared after radiation. However, metastasis occurred 14 months after presentation. Chemotherapy could not be used as the initial treatment because the patient’s condition was poor. Chemotherapy with cisplatin and etoposide were ineffective because of an adverse allergic reaction and poor general condition, but the lesions transiently diminished in size during the chemotherapy. According to previous reports, chemotherapy was effective for patients with germ cell tumor with metastatic extraneural lesions , 23. Che


Lung stereotactic ablative radiotherapy (SABR) has recently become more common in the management of patients with early-stage non-small cell lung cancer (NSCLC) and metastatic lung lesions who are not surgical candidates. By design, SABR is applied to small treatment volumes, using fewer but significantly higher dose fractions, and steep dose gradients. This treatment theoretically maximizes tumor cell death and decreases the risk of damage to the surrounding normal tissues. Local control rates for SABR in early stage lung cancer remain high. Since the numbers of primary tumor recurrences is small, some debate exists as to the appropriate definition of treatment failure. Controversies remain regarding the most appropriate interpretation of imaging tests obtained to evaluate treatment outcomes after lung SABR. Most definitions of progression include an increasing diameter of target lesion which can be problematic given the known mass-like consolidation seen on CT imaging after ablative therapy. Here, we present a case report illustrative of the pitfalls of relying solely on anatomic imaging to determine SABR treatment failure. The use of stereotactic ablative radiotherapy (SABR) has recently become more common in the management of patients with early-stage non-small cell lung cancer (NSCLC) and metastatic lung lesions who are not surgical candidates. Although the use of SABR continues to increase, controversies remain regarding the most appropriate interpretation of imaging tests obtained to evaluate treatment outcomes. Mass-like consolidation within the lung parenchyma after SABR can often mimic tumor progression making evaluation of patient outcomes difficult ,2. LikewA 70-year old male presented to our clinic after a chest computed tomography (CT) scan revealed a speculated mass in the anteriomedial portion of the left upper lobe of the lung. A review of the patient’s imaging studies from 1 year prior shows the speculated mass to be a new finding. The patient’s past medical history consisted of severe cardiopulmonary disease, end stage emphysema, aortic stenosis, prior myocardial infarction, advanced peripheral vascular disease and history of coronary artery bypass graft. The patient is a current smoker and reported a 90 pack-year history of cigarette smoking. He required 3 liters/minute of oxygen by nasal cannula 24-hours per day. The patient was registered in a prospective, IRB-approved registry designed to collect pertinent clinical, pathologic and treatment data on those patients receiving SABR at Mayo Clinic.18F] FDG Positron Emission Tomography (PET)/CT scan was performed for the purpose of staging and revealed a 3.3 cm hypermetabolic mass (SUV maximum 12.3) in the left upper lobe abutting the mediastinum . Treatment planning was performed with full body immobilization and 4-dimensional CT-based planning and daily cone-beam CT (CBCT) for image guidance. Since January 2008, a total of 372 patients have been treated with lung SABR and our institutional technique has been described previously [The patient returned to clinic approximately 2 months after completing SABR and a CT scan of the chest (2.5 mm slice thickness) showed significant reduction in size of the treated lung lesion without radiologic or clinical evidence of radiation pneumonits  of greater than 25% within the tumor region is termed progressive metabolic disease (PMD) , but no max measured on post-SABR PET imaging decreases in a linear fashion. This is shown in one recent study analyzing the SUV before and after SABR in patients with early stage NSCLC [max> 3.5 at 12 months post-treatment. Recently, authors from MD Anderson Cancer Center reported on the use of PET to assess local failure after lung SABR [In general, the SUVge NSCLC . Prior tOur patient is unique in that near complete response was seen on post treatment CT scans and all PET scans within the first 30 months after treatment were unremarkable for any FDG-avid lesions suggesting progression of disease. Another recent study of 80 patients receiving SABR for early-stage peripheral NSCLC reports that the median latency time for developing a mass-like consolidation in the area of treatment was 7 and 12 months in patients with and without tumor recurrence, respectively. Additionally, in all patients with tumor recurrence, consecutive rises in the volume of mass-like consolidation on serial imaging at 3-month intervals was seen . The devmax of ≥ 5.0 both obtained at least 6 months after completion of SABR is a reasonable definition for tumor progression. Either of these findings should warrant tissue confirmation with the caveat that the sensitivity of biopsy is not known for certain. One patient in a recent case series of surgical salvage following SABR, for example required 11 biopsy attempts for pathologic confirmation of recurrence [Given the typically short timeframe to recurrence seen after lung SABR as well as the high incidence of mass-like consolidation seen on follow-up imaging, one could potentially propose a new definition for tumor progression. Since tumor progression after SABR is an uncommon phenomenon and salvage treatment options are usually limited and associated with progressive risk, patience is warranted. Using the cut-off of three consecutive rises at 3-month intervals in the volume of mass-like consolidation as well as a two PET scans showing a SUVcurrence .Based on a recently published systemic review, a proposed algorithm for imaging follow up of SABR patients who are candidates for salvage treatment can be utilized . The patCurrent definitions of tumor recurrence after lung SABR may overestimate the true incidence of progression. The case presented here highlights the need to develop a definition which incorporates anatomic, functional and kinetic information to increase the diagnostic sensitivity and specificity of tumor recurrence after lung SABR.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.All authors declare that they have no competing interests.MCS drafted the manuscript and conceived the report. JWR participated in image acquisition and manuscript editing. MNW is the responsible clinic physician and edited the manuscript. YIG is responsible for the technical treatment design and follow-up recommendations on an institutional/ departmental level. KRO participated in coordination of clinical studies and drafting of the manuscript. All authors have read and approved the final manuscript.


Adult ureteroceles are generally known as simple ureteroceles with minimal obstructive effects 1 that can usually be managed endoscopically. Such pathology presented with acute abdominal pain and fever in a 32-year-old man with left renal agenesia, a cranial blind left ureter and left obstructed ureterocele. The retained secretions were suppurative. Adult ureteroceles generally known as simple ureteroceles with minimal obstructive effects are usually related to single systems They are intravesical and less obstructive and endoscopic interventions are proven effective for relieving symptoms . As statA 32-year-old man presented with sustained ongoing left lower quadrant (LLQ) pain aggravating three days before admission and fever. He had no gastrointestinal complaints apart from decreased appetite. Severe pain made walking impossible. He did not report any dysuria or obstructive lower urinary tract symptoms. On examination, there was LLQ tenderness without rebound or guarding, no costovertebral angle tenderness, a normal external genitalia and a low grade fever. Laboratory exam results were normal, urine analysis had one to two WBCs and one to two RBCs in the high power field (HPF). Before being referred to the hospital, he underwent abdominopelvic ultrasonography which revealed a normal right kidney, no free fluids and a normal looking spleen and liver. The left kidney could not be seen and there was a 55 × 65 mm2 solid-cystic mass postero-lateral to the left bladder wall. Abdominopelvic contrast enhanced CT did not show any stones in the urinary tract, there was left renal agenesis and a solid-cystic non enhancing pelvic mass behind the bladder .Office bOn admission, a transrectal sonogram revealed a normal prostate and bilateral seminal vesicles and a 56 × 62 mm2 sized cystic mass with significant wall thickness containing fluid and an ectopic hydronephrotic pelvic left kidney was inferred . The DMSAdult orthotopic ureterocele is a well-known entity, nevertheless concomitant blind ureteral bud, renal agenesis and ureterocele have only been reported in three cases previously, and a full-blown inflammatory and septic presenting feature of this case has led to a diagnostic challenge. Diverse differential diagnoses of a pelvic mass in the male which include prostatic cysts, mullerian duct remnants and malignancies of the seminal vesicles besides the scarcity of such cases in daily practice misled both the radiologists and the urologist. The patient was operated with a preoperative diagnosis of pelvic dysplastic kidney. Maas and colleagues reported the same cases being diagnosed by pelvic MRI  and anot


Jejunal diverticula are an uncommon acquired disease that is usually silent and asymptomatic. When symptomatic, they present with chronic nonspecific symptoms like pain, nausea, malnutrition and sometimes with acute presentation like gastrointestinal hemorrhage, peritonitis and obstruction. The majority of complications seen as an acute abdomen similar to appendicitis, cholecystitis or colonic diverticulitis but they also may appear with atypical symptoms. We are presenting a 63-year-old male reported in emergency with painful abdomen and diagnosed as having peritonitis. On laparotomy, we incidentally found giant and multiple jejunal diverticula along with ileal perforation. Nothing was done to the jejunal diverticula, as these were multiple and non-obstructive. In the follow-up of 16 months, the patient was doing well. Jejuno-ileal diverticulosis is a rare condition that continues to present formidable challenges in diagnosis and treatment. Although there is no consensus on the management of asymptomatic jejunal diverticular disease, some complications are potentially life threatening and require early surgical treatment [2]. Jejunal and jejuno-ileal localization is nearly three times less frequent than the duodenal, but of about four times likely to develop complications [2]. Asymptomatic cases require neither medical nor surgical treatment. Rarity of mild or chronic presentations explains the absence of clear consensus on therapeutic strategy and conservative management [2]. The majority of jejunal diverticulosis cases are discovered incidentally during radiological investigations. Surgical exploration is the treatment of choice for almost all acute complicated cases [2]. Most small bowel diverticula are thought to be acquired pulsion lesions. The clinical presentations of acquired jejunoileal diverticulosis are vague and diverse. As a result, identification of the disorder can be quite difficult. Presenting complaints such as intermittent abdominal pain, constipation and diarrhea, akin to those seen in irritable bowel syndrome, have been demonstrated in up to 90% of the patients and imaging tests have mostly atypical appearance without key diagnostic features and may not correlate with the clinical symptoms [1]. Thus, clinical recognition of the disease depends primarily on an awareness of the condition and the various ways in which it may present.Jejunal diverticulosis is a rare condition with variable clinical and anatomical presentations. The prevalence of small intestinal diverticula on autopsy ranges from 0.06% to 1.3% . Multiple diverticulosis of jejunum represents an uncommon pathology of the small bowel. The disease is usually silent and must be taken into consideration in cases of unexplained malabsorption, anemia, chronic abdominal pain or discomfort [5].Acquired jejunoileal diverticulosis was first described in 1794 by Sommering and later in 1807 by Sir Astley Cooper and is characterized by herniation of mucosa and submucosa through the muscular layer of the bowel wall  on the mesenteric border of the bowel [6] reviewed the literature and noted symptoms in 29% of the cases. Many symptoms may be misdiagnosed as dyspepsia or irritable small bowel. Edwards described a triad as 'flatulent dyspepsia' . Anemia due to iron deficiency and megaloblastic anemia have often been reported and commonly attributed to malabsorpion, steatorreia, and vitamin deficit [5]. Malabsorpion could be justified by the non-synchronous peristaltic movement of the bowel, the dilation of the diverticula, the stasis of the intestinal content and the bacterial overgrowth [7].Rodrigez et al. [1]. In the etiopathogenesis of the jejunoileal diverticula, the current hypothesis focuses on abnormalities in the smooth muscle or myenteric plexus. Careful microscopic evaluation of jejunal specimens with diverticula has shown that these abnormalities are of three types: fibrosis and decreased numbers of normal muscle cells, consistent with progressive systemic sclerosis; fibrosis and degenerated smooth muscle cells, suggestive of a visceral myopathy; neuronal and axonal degeneration indicative of visceral neuropathy. Any of these abnormalities could lead to distorted smooth muscle contractions of the affected small bowel generating increased intraluminal pressure. The result is herniation of mucosa and submucosa through the weakest mesenteric site of the bowel wall with penetration induced by paired blood vessels from the mesentery [5]. Jejunal diverticulosis is a challenging disorder from a diagnostic perspective, with no truly reliable diagnostic tests. Unfortunately, the only means of confirming a diverticulum source is by the cessation of the pain after surgical resection of the involved segment of small bowel. Asymptomatic jejunoileal diverticulosis does not require intestinal resection as seen in our case [8]. Today, multi-slice CT is very useful to make the diagnosis of jejunal diverticulosis and appears clearly superior to conventional enteroclysis for small intestine diseases [9].Abdominal or chest radiographs may demonstrate evidence of perforation, such as free air under the diaphragm or free peritoneal air, evidence of intestinal obstruction, or evidence of ileus, including multiple air-fluid levels and bowel dilatation. CT may identify thickening or inflammation of the jejunum or localized abscess formation [10]. Jejunoileal diverticulitis is quite uncommon and has a mortality rate as high as 24% [11]. We report a case of uncomplicated jejunal diverticulits in conjunction with ileal perforation and review its presentation, clinical features, and management. Complications requiring surgical intervention occur in 8%-30% of the patients [1]. In our case, no surgical intervention was required for diverticulum as it was giant, multiple in nature and non-obstructive.The complications of jejunoileal diverticulosis are chronic abdominal pain, malabsorption, hemorrhage, diverticulitis, obstruction, and perforation, and occur in 10%-30% of the patients [Jejunal diverticulosis in the elderly can lead to significant morbidity and mortality and so it should be suspected in those presenting with crampy abdominal pain and altered bowel habits. Once jejunal diverticulosis has been diagnosed, conservative medical management should be instituted to alleviate symptoms and reduce the risk of complications associated with diverticular disease. Jejunal diverticulosis in the elderly can lead to significant morbidity and mortality and so it should be suspected in those presenting with crampy abdominal pain and altered bowel habits. It should not be regarded as an insignificant finding and should be kept high in the differentials in older patients presenting with unexplained abdominal symptoms because it may lead to life-threatening complications and death. In the presence of complications, surgical resection with primary anastomosis is the preferred treatment option.


Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 revealed 333 nerve sheath tumors, of which 31 were MPNSTs. Four representative case reports are presented. Discussion. MNPSTs have rarely been reported from sub-Saharan Africa; in this study, they constituted 9.3% of all nerve sheath tumors. The trunk (42%) and limbs (45%) were the most frequently affected anatomical sites. Late presentation of malignant lesions in this environment is exemplified by the four case presentations patients. Conclusions. This report confirms observations from studies on MPNSTs from other environments. Anatomically centrally located MPNSTs may have a higher incidence in sub-Saharan Africa than in the West. Because NF1-associated MPNSTs are difficult to diagnose clinically, and because surgery is the only mode of therapy that offers a complete cure, a lifetime follow-up is important, as this would enable diagnosis of early lesions amenable to surgical extirpation. Benign and malignant peripheral nerve sheath tumors may occur sporadically or in conjunction with neurofibromatosis type 1 . MPNSTs Approximately 50% of MPNST occur in patients with neurofibromatosis type 1 . MPNSTs Factors associated with the development of MPNSTs include NF1 patients with many, extensive, or centrally located plexiform neurofibromas, as are nodular plexiform tumors associated with large peripheral nerve sheaths and extensive tumors involving the brachial, lumbar, or sacral plexus. Microdeletions of the NF1 locus may also be indicators of high risk for MPNST development , 10. MosThere is little reported in the literature on malignant peripheral nerve sheath tumors in Africa –15. AlthRecords on surgical specimens submitted for histological examination to the hospital pathology department between 1992 and 2008 reported as peripheral nerve sheath tumors were retrieved from the database. Clinical records of four patients who presented to the authors' hospital and were found to have malignant peripheral nerve sheath tumors were reviewed and are reported; these were not part of the data from the pathology department. An Internet/PubMed search of sub-Saharan published literature on MPNSTs was made.There were a total of 333 peripheral nerve sheath tumors diagnosed over the 16 years; 31 patients  had a histological diagnosis of malignant peripheral nerve sheath tumor . PatientThe authors found only four papers from sub-Saharan Africa that had reported MPNSTs –15.  A 21-year-old female patient with NF1 presented with lower limb pain and weakness for one year. She also had a large abdominal mass associated with early satiety and constipation for four months prior to presentation. She was cachectic, with a weight of 42 kg. A CT Scan showed an extensive retroperitoneal tumor compressing the diaphragm cranially, and filling the pelvic cavity, likely of paraspinal origin . At surgA 19-year-old female with NF1 presented with a one-week history of left thigh pain and inability to walk. She had multiple large bilateral upper limb masses  that were associated with pain and paraesthesiae . A skeleThe femur fracture was fixed; biopsies revealed metastatic MPNST. Subsequently, while still in the hospital, she developed bilateral lower limb weakness and stumbled and fell while walking. She sustained pathological fractures of the 4th and 5th lumbar vertebra and the right humerus. After operative fixation of the humeral fracture, she was referred elsewhere for chemoirradiation and got lost to followup.  A 35-year-old male presented with an ulcerated, pedunculated mass on the left thigh for a period of 5 years . He had A 21-year-old male with NF1 presented with a large painful, ulcerated mass involving the proximal two-thirds of the left leg . He had The patient was ambulant with crutches 24 hours after surgery, with minimal pain. Late presentation of patients with advanced malignancies is not uncommon in sub-Saharan Africa. Of the four representative patients, only one had a surgically curable lesion, even though he presented late with an ulcerated lesion; a second potentially curable lesion was the fourth patient who underwent an above-knee amputation. One of the other two patients had extensive bony lesions, resulting in a number of pathological fractures: femoral, humeral fracture, and lumbar vertebral fractures. The quality of life of the first and third patients improved significantly postoperatively. The rarity of MPNSTs in the sub-Saharan population is attested to by the few reports and low numbers from the region –15. OdebIn the current study, 9.3% of histologically diagnosed nerve sheath tumors were MPNST. One patient had a triton tumor; MPNSTs with rhabdomyoblastic differentiation may have a more aggressive clinical course than other MPNSTs . Sixty-oMPNSTs in other studies most commonly involve major nerve trunks, such as the brachial plexus, sacral plexus, or the sciatic nerve and often present with pain and motor or sensory deficits , 21. HowThe MPNSTs in multiple bones in one patient were either metachronous tumors or metastatic from the upper extremity lesions. Pathological fractures from MPNSTs are very rare but have been reported . Sar andPatients with MPNSTs may have a poorer prognosis than those with sporadic tumors, because NF1-associated MPNSTs tend to be centrally located, and therefore less amenable to surgery . Poor prAggressive wide resection (at least 10 cm margins), including limb amputation, is advocated for MPNST, as in the fourth case report. While this may be impossible in anatomically centrally located sites, unfortunately, surgery is the only modality of therapy that can achieve cure , 27, 28.While patients with NF1-associated MPNSTs presented at an average age of 27 years, those with sporadic MPNSTs had a mean age of 42 years, a finding consistent with other reports that have shown NF1-associated MPNSTs present at an earlier age . Retroperitoneal MPNSTs have a very poor prognosis, because of an insidious onset, local invasiveness, and frequently late diagnosis. Further, surgical excision is incomplete, and chemotherapy and radiotherapy are reportedly ineffective in retroperitoneal MPNSTs . PatientThere was no difference in the incidence of centrally located tumors between NF1-associated and sporadically occurring MPNSTs in the current study. This high incidence of nonextremity lesions in sub-Saharan Africa MPNSTs may be equated with poor prognosis, resulting from incomplete resections, recurrences, and deaths, especially in an environment where other modalities of therapy are either unavailable or unaffordable.The final outcomes of the 31 patients reported here are unknown. Of the four representative case reports, two returned for followup; the surgical margins in both patients were considered adequate, and adjuvant therapy was considered unnecessary. The outcome of the two female patients remains unknown as they did not return after oncology referrals. Because negative surgical margins were not achievable in the two patients and because of extensive metastases in one, it is likely that they may have succumbed to the disease and died. Oncologic services in Kenya remain either difficult to access because of large volumes of patients awaiting therapy in public institutions or inaccessible because of cost in private hospitals.Malignant peripheral nerve sheath tumors are rare malignancies that have been infrequently reported from sub-Saharan Africa. This clinicopathological report confirms observations from studies on MPNSTs from other environments: they are rare and 48% occurred in NF1 patients, in whom they occur at a younger mean age (27 years), in comparison to patients with solitary MPNSTs (42 years). Unlike most Western studies, however, there was no difference in the incidence of centrally located MPNSTs (which have a poor prognosis), between NF1-associated MPNSTs and solitary MPNSTs. Because malignant lesions in NF1 patients are difficult to diagnose clinically and because surgery is the only mode of therapy that offers a complete cure, a lifetime followup is important, as this would enable diagnosis of early lesions amenable to surgical extirpation.


Gastric metastasis from osteosarcoma is very rare and its clinical features are not well recognized.A 73-year-old man was diagnosed with osteosarcoma and treated with four cycles of preoperative chemotherapy with ifosfamide and doxorubicin followed by wide resection. Two cycles of postoperative chemotherapy with ifosfamide and doxorubicin and ten cycles of chemotherapy with carboplatin and etoposide were administered. Eleven months after the surgery, he vomited fresh blood. Unusual progression of anemia was observed with the hematemesis. A biopsy was performed by gastrointestinal endoscopy, and the stomach tumor was diagnosed as metastasis of osteosarcoma.Even though gastric metastasis from osteosarcoma is very rare, all three previous reports and our case showed the presence of ulcer on the surface of the gastric lesion. We should consider the possibility of gastric metastasis in patients with osteosarcoma in whom progression of anemia or gastric hemorrhage is observed. Osteosarcoma is the most common primary bone tumor in children and adolescents,2. But, 2 on days 1 to 5 and doxorubicin 20 mg/m2 on days1 to 3 every 3 weeks followed by wide resection which involved the sternum, bilateral costal cartilages, and pleura, chest wall reconstruction with mesh, and plastic reconstruction with a latissimus dorsi muscle free flap. Histological examination of the resected specimens revealed a necrosis rate of 95% and a microscopically negative surgical margin. Pleural effusion was observed after surgery, and fine needle aspiration cytology revealed tumor cells in the pleural effusion. Two cycles of postoperative chemotherapy with ifosfamide and doxorubicin and 10 cycles of chemotherapy with carboplatin 300 mg/m2 on day 1 and etoposide 100 mg/m2 on day 1 were administered every 4 weeks. No obvious lung or pleural metastasis was observed on chest computed tomography (CT) after these treatments. Eleven months after the surgery, blood tests showed a mild decrease of hemoglobin from 8.6 to 7.8 g/dl in one week, and he vomited fresh blood at the toilet of our hospital on the day of the blood test. Gastrointestinal endoscopic examination was performed promptly and showed a tumor on the gastric body that was like a type IIa + IIc gastric cancer  after neoadjuvant chemotherapy, with the most common primary recurrence site being lung (77.6%), followed by bone (8.3%), local (6.4%), local and bone (2.6%), lung and bone (1.6%), kidney (0.9%), brain (0.9%), and heart (0.9%)[et al. reported 64 cases with gastric metastases from primary sites including breast , lung , malignant melanoma , head and neck , and uterus , but none from osteosarcoma[Bacci  local 6.%, local n = 16, 2.0%, malin = 16, 2.0%, maliet al. reported that patients over the age of 60 years with high grade conventional osteosarcoma received less chemotherapy and fared worse than patients under the age of 60 years in a retrospective review of patients over the age of 40 years with osteosarcoma[et al. reported that neoadjuvant chemotherapy with ifosfamide, doxorubicin, and cisplatin for patients 41- to 60-years old yielded a similar clinical outcome to that of patients less than 40 years of age who were treated with conventional chemotherapy[Grimer osarcoma. Chemothotherapy. Since totherapy,12, we aet al. reported 17 gastric metastases in autopsies of 1,010 patients with cancers; 10 of these 17 did not have clinical manifestations[Menuck stations. Interesstations-7. In thstations,7. In ouVEGF expression in osteosarcoma was reported to be associated with a high potential for pulmonary metastasis. In our Summing up, we observed gastric metastasis from a trunk osteosarcoma in an older patient. Even though osteosarcoma gastric metastasis is infrequent, progression of anemia or gastric hemorrhage in the follow up of osteosarcoma should raise suspicion of a gastric metastasis.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.CT: Computed tomography;H & E: Hematoxylin and eosin;HSE: Hypertonic saline-epinephrine;VEGF: Vascular endothelial growth factor.The authors declare that they have no competing interests.HU designed and drafted the manuscript; YN developed the concept and treatment of the osteosarcoma; NI revised the manuscript and supervised; ST, EA, EK, and NF treated the osteosarcoma; IT and RM gave the endoscopic diagnosis; AH performed the surgical treatment of the gastric metastasis. All authors read and approved the final manuscript.