Adenoid Cystic Carcinoma of the lacrimal gland is a rare tumour. Their aggressive behaviour, with a high-risk of local recurrence, and late distant spread of the tumour even after aggressive management has been reported. Metastasis to the liver is rare and when it occurs, it is usually part of widespread metastasis, and therefore surgical treatment is seldom considered.We report a rare case of an isolated liver metastasis from a lacrimal gland adenoid cystic carcinoma 20 years after resection of the primary tumour. The patient presented with right upper quadrant pain radiating to the back and shortness of breath of 3 months duration. No local recurrence was detected during a 15 year follow-up with computerized tomography (CT) of the head. Abdominal CT scan demonstrated a solitary liver tumour with no other primary source, and the bone scan was normal. The patient was treated with an extended right hemihepatectomy. The histology revealed a predominantly cribriform tumour with focal areas of basaloid type metastatic lacrimal gland adenoid cystic carcinoma.This case illustrates the unpredictable behaviour of adenoid cystic carcinoma and the need for a life long follow up for these patients after treatment. The possibility of surgical resection for liver metastasis from adenoid cystic carcinoma should always be considered. Adenoid cystic carcinomas (ACC) of the lacrimal gland are rare malignant tumours accounting for 1.6% of all orbital tumours . DespiteThey are slow growing tumours, which tend to spread to adjacent structures and occasionally metastasise via haematogenous spread to lungs, brain and bone in decreasing order of frequency -8.There are few studies and reports on lacrimal gland ACC describing time interval to presentation of metastases and length of follow-up required ,10. The We present a case of an isolated liver metastasis occurring 20 years after initial surgery for lacrimal gland ACC, with no evidence of loco regional, synchronous or metachronous distant metastasis.In 1985, a 51-year-old male, underwent a radical craniofacial resection for a left lacrimal gland adenoid cystic carcinoma, followed by postoperative radiotherapy. He was followed-up with annual head and orbital CT scans for 15 years with no signs of loco-regional recurrence. Five years after discharge from follow-up, he presented with a 3-month history of right upper quadrant pain, radiating to his back, associated with shortness of breath and reduced exercise tolerance.Physical examination revealed hepatomegaly and right upper quadrant tenderness, but no stigmata of chronic liver disease. Head and neck examination revealed no sign of local recurrence. LFT, inflammatory markers, coagulation profile, CEA, CA 19-9, and AFP were all normal. An abdominal ultrasound (USS) examination and subsequent chest and abdominal CT scan showed a large 15 × 17 × 20 cm heterogeneous mass with a central area of low attenuation suggesting necrosis involving segments 4 to 8 of the liver  of the lacrimal gland account for 1.6% of all orbital tumours . DespiteACC in general has a slow biological growth and tends to have a protracted course with a poor outcome, with a reported survival rate of less than 50% at 5 years and 20% at 10 years -14. Lacret al, reviewed the outcome of 12 patients with low grade ACC, showing that all patients had local recurrence with a mean interval of 3.25 years, and a mean survival of 5 years [In 23 years retrospective study, Ramon  5 years . In the  5 years . In thisLate presentation of distant metastases is a recognised feature of ACC with reported distant metastasis rates of 19–24%, and a mean time to presentation of 7.6 years . RecurreMetastasis to the liver is rare and usually only occurs in conjunction with more widespread metastatic disease . ReporteThis patient had liver metastasis as the sole presentation of secondary disease 20 years following treatment for his primary with no evidence of local recurrence. Surgical resection of liver metastasis from lacrimal gland ACC has never been reported before.There is no proven effective chemotherapy for the treatment of metastatic lacrimal gland ACC; however some studies suggested benefit from neoadjuvant and adjuvant chemotherapy in the treatment of primary lacrimal gland ACC, in terms of improving local disease control and overall disease free survival .This case highlights new aspects on ACC of the lacrimal glands, and a new evidence of the unpredictability of ACC behaviour in general.Solitary hepatic metastasis can occur extremely late after resection of lacrimal gland ACC. Life long follow-up of patients with ACC is mandatory as treatable single organ recurrence may occur decades after resection of the primary. Resection of such liver metastasis is possible and should be considered.The author(s) declare that they have no competing interests.BAZ, MAH the main operators in charge of the idea, literature search, the manuscript, article drafting and formatting.MAG literature search and figures preparation.HEM histological examination.NWP performed surgical operation, critical revision of the article.JNP critical revision and final approval of the version.All Authors read and approved the final version of the manuscript


Although pheochromocytoma occurs in 1% of patients with von Recklinghausen's disease, composite tumors in this syndrome are much rarer, with isolated case reports in the literature. Most gastrointestinal stromal tumors (GISTs) are solitary and sporadic. Multiple GISTs however, are associated with clinical syndromes particularly von Recklinghausen's disease. We believe this is the first report of composite adrenal pheochromocytoma and multiple GISTs occurring in an 82 year old woman with neurofibromatosis type 1 (NF1), manifested by clitoral and subcutaneous neurofibromas, epilepsy and Lisch nodules. The extreme clitoromegaly raised concerns of pseudohermaphrodism at presentation. Neurofibromatosis type 1 (NF1) is a heterogeneous, dominantly inherited disorder associated with a large number of different neoplasms, both benign and malignant. Pheochromocytoma occurs in 1% of patients with NF1, whereas composite tumors of the adrenal medulla are much rarer. By definition, a composite tumor consists of pheochromocytoma with neuroblastoma, ganglioneuroblastoma or ganglioneuroma. Both cell lines, the chromaffin cells and the symapathetic ganglion cells are of neural crest origin. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract and have also been described in association with NF1. GISTs are immunohistochemically positive for KIT tyrosine kinase receptor and this characteristic is also found in the interstitial cells of Cajal, the pacemaker cells regulating autonomic motor activity. It is postulated that GISTs originate from a multipotential precursor cells, which differentiate to an interstitial cell of Cajal phenotype. Neurofibromas of the clitoris have been reported infrequently. It represents a differential diagnosis that needs to be considered in pseudohermaphrodism.NF1 has been associated, albeit rarely, with composite ganglioneuroma-ganglioneuroma of the adrenal medulla, multifocal gastric GISTs and neurofibroma of the clitoris, but we believe this is the first case report whereby all these disorders occur in the same patient with NF1.An 82-year old widow and mother of three with neurofibromatosis was hospitalized following a seizure due to non-compliance of anticonvulsant medication Her epilepsy had been previously well controlled on phenytoin 300 mg daily. Cranial computed tomography (CT) revealed an old left frontal cerebral infarct. The patient's other past history included mild aortic stenosis, fractured left neck of femur and iron deficiency anemia. Gastroscopy and colonoscopy were performed 3 years previously, when she was investigated for melena and no obvious cause found. Glibenclamide 5 mg tds had been commenced by her general practitioner for symptomatic glycosuria. She was on no other medication.During this admission, the patient developed a gastrointestinal pseudo-obstruction, which was managed conservatively. It was during the investigation of her constipation and progressive abdominal distension that clitoromegaly was noted on examination and a 2.5 cm left adrenal mass found on abdominal CT.The patient gave a history of clitoral enlargement from birth and recalls clitoral reduction surgery being offered and declined at 4-years of age. She developed numerous subcutaneous nodules at 16-years of age and cutaneous neurofibroma was confirmed on biopsy of the skin nodule at that time. There was a family history of neurofibromatosis, which afflicted both her maternal grandmother and great-grandmother, and the patient's own youngest daughter.Given the association of neurofibromatosis and pheochromocytoma, the patient was questioned regarding paroxysmal symptoms of palpitations, headaches, sweats, flushing or visual disturbance, but these were absent. On examination she was normotensive with a postural drop from 135/60 mmHg to 110/60 mmHg. The pulse rate was 80 and regular and there was an audible, soft, aortic stenotic murmur. Widespread cutaneous neurofibromas and café-au-lait patches were noted. Clitoromegaly was present with the clitoris measuring 10 × 4 × 4 cm. The visual fields and fundi were normal although Lisch nodules were present on the left iris. The patient had bilateral sensineural hearing loss but no other neurological deficits were detected.Biochemical investigations confirmed elevated 24-hour urinary catecholamine excretion 6 days post admission: adrenaline 232 nmol/day , noradrenaline 465 nmol/day (ref. <600); and 24 hr urinary creatinine 4.6 mmol/day (ref. 5.0–16.0). A repeat 24 hr urinary catecholamine three days later also confirmed persisting elevation of urinary adrenaline of 149 nmol/day with urinary creatinine of 4.4 mmol/day. Urinary excretion of cortisol was 530 nmol/day (ref. <590). The patient was biochemically euthyroid with free thyroxine of 13 pmol/L (ref. 9–19) and TSH 3.15 mU/L (ref. 0.30–4.00). Prolactin was 380 mU/L (ref. <420) and she was normocalcemic, total plasma calcium corrected to albumin of 40 g/L was 2.47 mmol/L (ref. 2.25–2.60) and ionised calcium 1.18 mmol/L (ref. 1.12–1.32) adjusted to pH of 7.4. The serum sex hormone profile revealed testosterone 3.1 nmol/L (ref. <3.5), estradiol 110 pmol/L (ref. 40–130), free androgen index 2.3 (ref. 1–5), DHEA-sulphate <0.2 μmol/L (ref. 0.4–2.4), and androstenedione 4.4 nmol/L (ref. 1.6–9.3). Her serum 17-hydroxy progesterone was mildly elevated at 2.3 nmol/L (ref. 0.3–1.8) but this was felt to be of no clinical significance. Cytogenetic assessment of peripheral blood revealed a normal female karyotype.123I-MetaIodoBenzyl Guanidine (MIBG) scintigraphy. The patient was prepared for surgery with phenoxybenzamine 30 mg bd one week pre-operatively with propranolol 40 mg bd added the day prior to surgery. Laparoscopic left adrenalectomy was undertaken, but this was converted to an open adrenalectomy due to technical difficulties. During this procedure, approximately ten nodules measuring between 3 and 5 mm were noted on the serosal surface of the stomach, one of which was biopsied. Inspection of the rest of the intra-abdominal viscera was unremarkable. Core biopsies of the enlarged clitoris were taken. The post-operative course was uneventful and the patient made an excellent recovery. Repeat urinary catecholamine excretion prior to discharge was normal.Given the persistently elevated urinary adrenaline excretion, the CT finding of a 2.5 cm left adrenal tumor was likely to be functional and this was confirmed using At two-month follow-up after her adrenalectomy, a clitorectomy was performed when a painful ulcer developed on the clitoris.Macroscopically, the biopsy consisted of an irregular piece of firm pinkish tan tissue measuring 6 × 5 × 4 mm. Microscopically, there was a lobulated tumor composed of elongated uniform spindled cells separated by collagenous stroma. The nuclei were arranged in palisades and there was no cytological atypia and no mitoses were present. Small foci of tumor extended into the adjacent smooth muscle. Immunohistochemistry showed strong and diffuse staining for c-kit (CD117) and CD34. There was also focal staining for desmin and smooth muscle actin. There was no staining with S100. The findings were consistent with a small gastrointestinal stromal tumor (GIST).The biopsy consisted of a core of soft, whitish grey tissue measuring 9-x-7x-1.5 mm and the entire tumor consisted of a skin covered polypoid mass measuring approximately 90 × 40 mm. There was a large kidney-shaped ulcerated area with well defined whitish margins measuring 43 × 30 mm. Microscopically there were small circumscribed nests of neural type structures reminiscent of sensory Meissnerian corpuscles separated by slightly myxoid stroma containing numerous mast cells. Focally, there was a diffuse architectural pattern of ill-defined fascicles of cytologically uniform spindle cells with abundant fibrillary matrix. Immunohistochemistry showed S100 positivity. Histological diagnosis was a clitoral neurofibroma.The left adrenal gland measured 48 × 25 × 50 mm and weighed 11 grams. Bulging above the surface was a nodule measuring 20 × 20 × 13 mm, which was variegated grey-yellow-orange on the cut surface. A 6 mm greyish nodule was found within the adjacent fatty tissue. Microscopically, the majority of the tumour (70%) showed typical features of a pheochromocytoma, with discrete nests of tumour cells separated by delicate fibro-vascular septa. These cells were of large with conspicuous granular cytoplasm and showed pleomorphism, many cells having distinct nucleoli. Mitotic figures were not seen and there was no necrosis. The cells extended through the capsule focally into the peri-adrenal fat. No vascular invasion was seen. In a few areas the tumour cells were more widely separated by thick bands of collagen. The second component of the tumour (30%) showed features of ganglioneuroma, with large mature ganglion-type cells with large nuclei, central eosinophilic nucleoli and abundant cytoplasm embedded within a fibrillary neural meshwork containing cytologically uniform spindle-shaped cells. No primitive neuroblastic elements were seen. The two tumour components were well demarcated in some areas, but focally appeared admixed.Immunohistochemistry showed no staining of the tumour elements with the proliferation marker Ki-67 or p53 protein. Positive staining for S100 protein was identified in the sustentacular cells within normal adrenal medulla and in the phechromocytoma, as well as in the ganglion cells of the ganglioneuroma. The adrenal tumour was therefore a mixed pheochromocytoma/ganglioneuroma or composite pheochromocytoma.The nodule noted adjacent to the main adrenal swelling consisted of an enlarged ganglion including expanded and distorted nerve fibres. The appearances suggested focal involvement by neurofibroma.Neurofibromatosis type 1 (NF1) is one of the most common dominantly inherited disorders, with a birth incidence of 1 in 3000 . The NatExpression of NF1 is highly variable. Patients with this disorder have an increased incidence of both benign and malignant tumours. This was shown in an investigation of 70 adult NF1 patients in Sweden . NF1 is Composite adrenal medullary tumours, on the other hand, are very rare. In an analysis from the National Cancer Institute of 120 adrenal and extra-adrenal Pheos, only four had a composite phenotype, making an overall incidence of 3% . A revieBolande  was the Sympathogonia derived from certain neural crest cells differentiate into neuroblasts and pheochromoblasts. These neural crest-derived cells migrate ventrally and differentiate to give rise to the cellular components of the sympathetic ganglia and the adrenal medulla. Any migration disturbance or maldevelopment of the neural crest might result in the development of composite tumours. As these cell populations have similar derivation one might expect composite adrenal medullary tumours to be more common than what is apparent from the literature and this may represent under-reporting or under-recognition of this condition.NF1 affects the gastrointestinal tract in 10–25% of cases  and it dGenitourinary neurofibromas are rare and clitoral involvement in NF1 has been reported infrequently. However, when it occurs, clitoromegaly is often the presenting sign. In many cases it is congenital. In a study of 236 families with NF1, four patients had clitoral involvement and in three, involvement was limited to the clitoris . A revieThis case report illustrates the heterogeneity of NF1 with the concomitant presentation of three rare manifestations of this fascinating disorder, namely composite pheo-GN of the adrenal medulla, multiple gastric GIST and neurofibroma of the clitoris. To our knowledge this is the first time such a case has been reported in the literature.


Tumor spread beyond the peritoneal cavity in cases of papillary serous adenocarcinoma of the unknown primary (CUP) is a rare late event and carries a poor prognosis.A 71-year-old female was referred to our hospital because of a large right inguinal tumor with biopsy evidence of carcinoma as well as an elevated serum CA125 (cancer antigen 125). She underwent complete resection of the right inguinal tumor and multiple pelvic tumors, which involved the rectum, ovary and uterus. Pathological examination revealed the tumors to be metastases of a papillary serous adenocarcinoma with a psammoma body of CUP. On the 28th postoperative day, newly developed asymptomatic small left inguinal node metastases in the setting of a normal CA125 level were removed. Four and a half years after the primary resection, the CA125 level increased again and newly developed asymptomatic metastases were found in the right deep inguinal nodes and extirpated at that time. All surgical resections followed the modified FAM  regimen, including protracted dairy oral administration of UFT or 5'-FDUR, Cimetidine and PSK (protein-bound polysaccharide K) as an immunomodulator or biological response modifier in conjunction with intermittent one-day continuous infusion (ADM+MMC) or intermittent single bolus injection of ADM+MMC. At present, the patient has been living in good health for almost 7 years with no evidence of relapse.Aggressive resection surgery followed by effective adjuvant chemotherapy is necessary for surviving long time without relapse of poorly prognostic patients with metastases outside of the abdominal cavity from peritoneal papillary serous adenocarcinomas. Despite recent advances in diagnostic imaging and molecular pathology, CUP accounts for about 3 % of all malignant neoplasms, which represent a group of heterogeneous tumors sharing unique clinical characteristics of metastatic epithelial cancers with no identifiable origin even on autopsy . The hetHerein, we present a female patient with repeated bilateral inguinal lymph node metastases from peritoneal papillary serous adenocarcinomatosis of CUP, who is alive and well almost 7 years after the initiation of therapy involving multiple resection surgeries and unique adjuvant chemoimmunotherapy.A 71-year-old female was admitted to our hospital with a large right inguinal tumor (5 cm in diameter) on June 2, 1999. She complained of inguinal mass increasing in size for the 4 months preceding admission. She had a past history of an appendectomy for acute appendicitis 16 years previous to admission. No abnormalities were found in her complete blood and biochemistry profiles. Serum carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA19-9), squamous cell carcinoma antigen (SCC) were all within normal limits, but CA125 was elevated to 360 U/ml at hospitalization. Ultrasonography, CT-scan and MRI demonstrated not only a right inguinal tumor, but also showed large pelvic tumors Fig. &1B. The CT on the 23rd postoperative day demonstrated new small asymptomatic inguinal tumors adjacent to the left external iliac artery. The diameter of those tumors was 15 mm and 12 mm, respectively. Those tumors were extirpated completely on July 29, 1999.The right inguinal tumor, approximately 4.5 × 5 × 4 cm in diameter, was relatively well circumscribed by the right inguinal ligament, and right external iliac artery and vein, which are supplying and draining tumor blood flow. After extirpation of the inguinal tumors, the intra-pelvic tumors were removed completely by total hysterectomy with salpingo-oophorectomy and a partial resection of the rectum at the rectosigmoid junction Fig. &1D. TherGrossly, the right inguinal tumor was elliptical and well circumscribed, measuring 42 × 25 × 12 mm in size. The cut surface of the solid tumor was swollen and reddish-white, with hemorrhagic and necrotic foci Fig. . Histolo2 receptor antagonist; 600 mg/day) and PSK (3 g/day) was started. We used Cimetidine not only as an anti-ulcer drug, but also as an immunomodulator or biological response modifier with the hope of eradicating possible microscopic residual cancer cells [®) to the superior vena cava by way of the right subclavian vein. A portable elastomeric infusion system filled with 50 ml saline dissolution of Mitomycin C  and Adriamycin  was applied every other week at an outpatient clinic. Three months after we changed from UFT to 5'-FDUR (600 mg/day) because MMC may be more effective with 5'-FDUR (due to stronger inductive activity of thymidine phosphorylase (dThdPase) in tumor tissues [Her postoperative course was uneventful, except for the early detection of the left inguinal node metastasis by CT on the 23rd postoperative day. This tumor was extirpated promptly and postoperative imaging procedures confirmed that no tumor remained anywhere in the body. Pathologic examinations revealed lymph node metastasis from a papillary serous adenocarcinoma Fig. . Preoperer cells . PSK waser cells ,10. In o tissues ,12.The implanted port and access catheter were removed two years later. The serum tumor markers CA-125, CEA and CA19-9 were checked every month and image diagnostic screening by CT and US were carried out every three months during 4 postoperative years, however, no suggestive signs of relapse had been found anywhere in the body. However, from 4.5 years after the last surgery, the serum level of CA-125 had gradually elevated to 140.5 U/ml and the CT scans demonstrated newly developed asymptomatic single tumor growth (25 × 20 mm) on the right deep inguinal node Fig. , though Despite modern imaging technology, including Positron Emission Tomography (PET) scanning, the primary site remains unknown in patients with metastatic CUP, even on autopsy. Metastatic CUP represents a heterogeneous group in terms of clinical presentation, histology and presumably biology. Lymph node involvement, the number of metastatic organ sites, hepatic involvement, and tumor histology have been reported as significant independent prognostic factors . AlthougFemale patients with metastases to the inguinal nodes from peritoneal papillary serous carcinomas of CUP are rare and have poor prognoses. There is no standard effective chemotherapeutic regimen for these patients. However, complete tumor removal followed by appropriate chemotherapy can lead to long-term survival even if the patient had nodal metastases beyond the abdominal cavity. Our modified FAM using protracted intermittent infusion of low dose MMC/ADM combined with oral UFT/5'-FDUR with PSK/Cimetidine may prove to be an efficient chemotherapeutic regimen with low toxicity, and which is amenable to good compliance.


A case of Renal Cell Carcinoma (RCC) presenting to the Emergency Department with pyrexia and rigors is discussed. A 61 year old female patient presented to the Emergency Department with feeling unwell, pyrexia, nausea and headache. She gave a history of fever for the past three weeks with three episodes of rigors. She also gave a history of recent loss of appetite and some weight loss. She had no urinary problems.On examination she looked pale. A mass was palpable in the right lower quadrant and lumbar region. Liver and spleen were not palpable. There were no signs of peritonitis.Her pulse rate was 125/minute, BP 120/85 and temp 39.6 degree Centigrade. She had blood tests in the Emergency Department which revealed the following : Hb 12.0 g/dL, WCC 11.6 × 10(9)/litre and Platelets 237 × 10(9)/litre. Electrolytes and urea were as follows: Na 133 mmol/litre, K+ 4.2 mmol/litre, Urea 5.1 mmol/litre and Creatinine 78 micromol/litre. Chest X ray did not reveal any abnormality.The patient had Ultrasound Scan which showed mass in the right Kidney Figure . Her subShe underwent right sided nephrectomy after full staging procedures and appropriate investigations.Malignant neoplasms involving the kidney may be primary or secondary tumors. Although metastatic lesions outnumber primary tumors, secondary renal neoplasms are usually clinically insignificant and are principally discovered at postmortem examination.Patients with Renal cell carcinoma (RCC) present with a range of symptoms, but many are asymptomatic until the disease is advanced. At presentation, approximately 25 percent of individuals either have distant metastases or significant local-regional disease. Other patients, even some with only localized disease, present with a wide array of symptoms and/or laboratory abnormalities. Because of this unusual characteristic, RCC has been labelled the "internist's tumor". Today, The classic triad of flank pain, hematuria and flank mass is uncommon 10% cases) and is indicative of advanced disease. The frequency with which Renal Cell Carcinoma clinically presents is shown in table 0% cases Renal cell carcinoma represents a heterogenous group of tumors, the most common of which is clear cell adenocarcinoma. RCC accounts for 3% of adult tumors. The incidence has increased more than 30% over the past two decades. It is generally accepted that the increased incidence rates reflect earlier diagnosis at an earlier stage, largely due to more liberal use of radiological imaging techniques. However advanced disease has also been diagnosed more frequently and mortality has increased as well .Symptomatic presentation correlates with aggressive histology and advanced disease. Incidental tumours may be frequently detected in female and elderly patients, as these groups traditionally seek general medical care more regularly. The mode of presentation can independently predict an adverse patient outcome. Indicators of symptomatic presentations include flank pain, flank mass, varicocele, constitutional symptoms, paraneoplastic syndromes and bone pain related to metastatic disease .Ultrasound scan was found to be useful screening test, but CT is the imaging study of choice to identify malignant features. MRI can be used in equivocal cases .Pre-operative clinical variables may be used instead of the pathologic stage to determine the risk of recurrence .Renal cell carcinoma presents with various clinical features. Atypical presentations of RCC should be considered for patients presenting with pyrexia of unknown origin.The author(s) declare that they have no competing interests.DD wrote the first draft of the manuscript and scanned photographs for submission. MS proofread the case report and obtained patient consent. NS performed the literature search.


Parathyroid carcinoma is a rare malignancy, with an incidence of 0.5 to 4% of all cases of primary hyperparathyroidism. Surgery is the only curative treatment.We report the case of a 66-year-old man referred for a large suspicious substernal goitre associated with severe hypercalcemia due to hyperparathyroidism. After normalization of serum calcium levels, patient underwent surgery. The voluminous cervicomediastinal firm mass could not be removed through the cervical incision; therefore a cervicothoracic approach was employed. Histopathology revealed a giant parathyroid cancer of 450 grams. A review of the literature was also undertaken to summarize the current treatment approaches for this rare malignancy.En bloc resection of the tumour with the adjacent tissue is the treatment of choice and it is very important to avoid the rupture of the capsule during operation. Neither tumour size, nor the lymph node status appears to play a role in prognosis. The management of parathyroid carcinoma is a challenge even for experienced surgeons.Parathyroid cancer is usually not recognized either preoperatively or intra-operatively.  Parathyroid Carcinoma (PC) is a rare malignancy with an incidence of 0.5 to 4% in all patients surgically treated for primary hyperparathyroidism ,2. GenerWe report the case of a patient admitted to our department for a large substernal goitre suspected to be a thyroid cancer, associated with severe hyperparathyroidism.A 66-year-old man was referred with a diagnosis of hyperparathyroidism, mild renal failure, peptic ulcer and large suspicious cervico-mediastinal goitre. The patient had fatigue, anaemia, anorexia and bone pain for several months. The fine needle aspiration of the neck mass, performed before the admission showed the presence of malignant cells. Preoperative laboratory findings are reported in table Ultrasonography of the neck showed an enlarged thyroid gland with substernal extension; many solid nodules and some calcifications were present. The computerised tomographic (CT) scan showed a large solid mass in left neck, with extension to the mediastinum, and displacement of the trachea, oesophagus and great vessels Figure . TracheoTc-99m sestamibi scintigraphy demonstrated diffuse radiopharmaceutical uptake within the thyroid region and the lower portion of the neck. This tracer accumulation corresponded to the mass and it was persistent during the different phases of the scintigraphy without significant changes Figure . The lacThe patient was vigorously hydrated and bisphosphonates were given preoperatively to control hypercalcemia. Eight days later the serum calcium level decreased to 10.4 mg/dl. With the above clinical findings, the patient underwent surgical neck exploration.en bloc resection of the mass along with the total thyroid gland and the connective tissue apparently involved in the tumour was performed. All the lymphoid tissue from the ipsilateral tracheoesophageal groove to the upper mediastinum was removedAt surgery a voluminous cervicomediastinal firm mass was found that could not be removed via the superior thoracic strait; therefore a complete sternotomy was performed. The surgical findings revealed an ovoid, grayish-yellow, hard mass, measuring 12 × 9 × 8 cm, adherent to the base of the left lobe of the thyroid, extending to the anterior upper mediastinum; the recurrent laryngeal nerve was laterally displaced but not invaded. Therefore The histological report described a parenchymatous thyroid gland, with a subcapsular gelatinous nodule (1 cm diameter). The voluminous tumour adherent to the base of left lobe was multinodular, greyish-yellow. Histologically many fibrous bands of acellular collagenous tissue extending from the thickened capsule, subdivided the neoplasm into irregular compartments. The tumour cells, arranged in trabecular and solid sheets, were homogeneous; they showed round – ovoid nuclei, with evident nucleoli, and clear cytoplasm; occasionally cytoplasm was abundant, eosinophilic and granular; nuclei were pleomorphic; mitoses were present (3 per 10 HPF). Rare multinucleated osteoclastic-like giant cells were found around foci of haemorrhage; necrosis and coarse calcifications were observed. Both capsular and vascular invasion were present at the periphery of neoplasm. In the infiltrated adjacent adipose tissue, well-circumscribed, encapsulated nodules of tumour, with a peripheral rim of lymphoid tissue were found; they were considered to be metastatic lymph nodes. Final diagnosis was a parathyroid carcinoma Figure . The posThe patient also had severe postoperative hypocalcaemia, necessitating calcium and vitamin D therapy. The postoperative laboratory values at hospital discharge are shown in table At follow-up, three, six, nine and 12 months after surgery, the patient was disease-free with normal levels of PTH and calcaemia and improvement of the bone disease symptoms. Whole body positron emission tomography (PET) performed one year after surgery was negative.Parathyroid carcinoma is a rare disease and the preoperative diagnosis is often difficult because clinical manifestations are similar, although more often severe than in patients with parathyroid adenomas. Parathyroid carcinomas are generally larger and firmer than parathyroid adenomas. The clinical manifestations of hyperparathyroidism are usually more severe, and serum levels of calcium, PTH and alkaline phosphatase are significantly higher than in patients with adenoma. Patients with parathyroid carcinoma have a high incidence of renal dysfunction, osteoporosis and gastrointestinal symptoms ,2,4.en bloc resection with ipsilateral thyroid lobectomy and removal of any enlarged or abnormal lymph nodes. Most of the authors emphasize the importance of an aggressive initial approach in reducing local and distant recurrences [Preoperative and intraoperative suspicion is important in planning the effective surgical treatment: the complete resection of the tumour at the first neck exploration is reported to provide the best chance of cure. The optimal surgical treatment is urrences ,6-12. Paurrences ,13; in turrences ,15.et al, have recently reported a case of resection of multiple PC lung metastases 20 years after the initial neck surgery, with a significant improvement of clinical and metabolic complications after removal of metastatic parathyroid cancer from both lungs [The management of the recurrent laryngeal nerve is more controversial but it should be resected only when it is not functioning preoperatively or is invaded by tumour ,4,15,16.th lungs .Surgery is also important to palliate hypercalcemia-associated metabolic complications and symptoms and to prolong survival ,15,20,21Recently some authors have reported a role for adjuvant radiation therapy. Neck and mediastinum adjuvant irradiation has been reported to be useful in reducing the risk of loco-regional disease progression and in improving survival ,22-24. OIn our case, although the laboratory data revealed marked hyperparathyroidism and the patient had all the signs and symptoms of severe hyperparathyroidism, none of the preoperative localizing studies raised suspicion of the presence of a parathyroid carcinoma. The slow growth of the mass and the preoperative FNAB suggested a large substernal goitre with malignant transformation; only the postoperative histological exam revealed a diagnosis of parathyroid cancer.en bloc" resected and the patient was alive and disease free after 4 years [This is an interesting case because of the large size of the parathyroid cancer with regional nodal metastases without any evidence of distant metastases or local recurrence one year after initial surgery. In the literature parathyroid cancers ranges from 1.5 to 6 cm in diameter and from 1.5 to 27 grams in weight ,26. A ca 4 years ,28. Our  4 years . The out 4 years ,29.Based on current knowledge, clinicians should consider parathyroid carcinoma in differential diagnosis when patients present with severe hyperparathyroidism and a palpable cervical mass. When there is an intraoperative suspicion of malignancy, a complete surgical resection should be undertaken with special attention to avoid the rupture of the capsule of the tumour. We believe this approach offers the best chance for a successful outcome.The author(s) declare that they have no competing interests.MGC Conceived the study, carried out the literature search, and draft the manuscriptFS SL UM helped in management of the patient and preparation of the manuscriptSL Carried out the preoperative scintigraphy and helped in preparation of the manuscriptLP Shaped the idea for the manuscript, coordinated the study and edited the manuscriptAll authors read and approved the final manuscript.


Reactive lymphoid hyperplasia (RLH) of the liver is very rarely reported, and we encountered two cases of RLH of the liver in a patient with colon cancer.In the first case, a 77-year-old woman was admitted for the surgical removal of a ascending colon cancer. A hepatic tumor in the left lobe was concurrently revealed by computed tomography (CT), and magnetic resonance imaging (MRI). The appearance suggested liver metastasis. Right hemicolectomy and partial hepatectomy were performed. On histopathological examination, lymphoid follicles with germinal centers were seen in the tumor-like lesion, and remarkable lymphoid infiltration with germinal centers was seen in the portal area around the nodule. Immunohistochemical studies revealed polyclonality of infiltrating lymphocyte. Consequently, this nodular lesion was diagnosed as RLH of the liver. In the second case, a 64-year-old woman who had a radical right hemicolectomy for stage II ascending colon cancer 10 years ago was admitted with dysuria. A hepatic tumor in the left lobe was concurrently revealed by CT and MRI, suggesting hepatocellular carcinoma. A left lateral segmentectomy was performed. Microscopically, this lesion revealed the almost same findings as the first case, so this nodular lesion was diagnosed as RLH of the liver.Our two cases were the first report of RLH of the liver accompanying colon cancer. Because there are a very few cases, so it is not clear whether the malignancies were involved in the onset of RLH. But we believe that new factors involved in the onset mechanism of RLH may be identified by carefully monitoring the clinical course of our two patients. Reactive lymphoid hyperplasia (RLH) is reported to occur in the gastrointestinal tract , skin 22, lung , thyroidA 77-year-old woman was admitted to our hospital with right upper abdominal pain in December 2004. Barium enema and colonoscopy revealed an ulcerative tumor in the ascending colon, which was diagnosed as moderately differentiated adenocarcinoma on biopsy. Physical examination showed no remarkable abnormalities. Neither hepatomegaly nor splenomegaly were apparent. Serum hepatitis B surface antigen and hepatitis C antibody were negative, and she had no history of alcohol intake or blood transfusion. Laboratory data on admission including liver function tests were unremarkable. Carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and α-fetoprotein (AFP) were within normal ranges. Abdominal ultrasonography showed a hypoechoic, 1.5-cm diameter mass in the left hepatic lobe (S3). Computed tomography (CT) revealed a low-density area in the same area, the interior of which enhanced equally. The outline of the mass was particularly enhanced in equilibrium phase . Plain CT examination revealed a low-density area, the mass was enhanced by the contrast medium in early phase. At a late phase, the mass was showed as a low-density area. MRI demonstrated a slightly low-intensity lesion on T1-weighted images and a high-Intensity on T2-weighted images. SPIO uptake was not detected on T2-weighted images. At an early phase, Dynamic MRI revealed low-intensity mass in S2 Fig. . At a laCase 1, so this nodular lesion was diagnosed as RLH of the liver, too. No recurrence has occurred during 10 months of postoperative follow up.Macroscopically, the resected liver had smooth surface and a normal appearance, cut section of the resected liver showed a well-circumscribed, not encapsulated, whitish mass in S2 of the liver Fig. . It measReactive lymphoid hyperplasia is recognized as a region that is well demarcated and characterized by hyperplastic lymphoid follicles with reactive germinal centers, which consist of polymorphic and polyclonal cell populations and aggregations of mature lymphocytes, plasma cells, and other types of inflammatory cells . In the By previously reported cases, in terms of imaging findings, RLH of the liver is depicted as a hypoechoic lesion on ultrasound and as a low-density lesion on plain CT, but contrast CT findings vary. Both MRI T1-low and T2-high intensity images yield the same results, and angiography shows hypervascularity. In our patients, results of preoperative diagnostic imaging conformed to this pattern; i.e. low-echo and plain CT demonstrated a low-density lesion, while contrast CT showed mild enhancement. Moreover, on equilibrium phase of contrast CT in the first patient, the area around RLH was depicted as a high-density area, possibly indicating a capsule. The present study was the first to conduct contrast SPIO-enhanced MRI in hepatic RLH. No SPIO uptake into the mass occurred, and the boundary with the normal liver was very clear. The further discussion of such rare cases based on the imaging findings including SPIO-enhanced MRI will provide more information about this rare entity. But, at this point in time, it is very difficult to completely rule out malignancies such as HCC, metastatic liver tumors, or malignant lymphomas based solely on diagnostic imaging findings. Ultimately, preoperative biopsy is therefore an essential diagnostic tool.While the cause of RLH has not been clarified, most patients are middle-aged or elderly women. In terms of underlying diseases, various chronic inflammatory diseases, such as chronic hepatitis, Sjogren's syndrome, chronic thyroiditis, and primary biliary cirrhosis, have been confirmed. Hence, autoimmune mechanisms are most likely involved. In our cases, the patients were a middle-aged woman and lymphoid infiltration was observed in the portal area around the nodule, suggesting a similar mechanism. However, lymphoid infiltration was confined to the area around the nodule, the rest of the hepatic parenchyma was normal, and hepatitis virus markers were negative, suggesting that this lesion was local rather than diffuse. Furthermore, as systemic immunologic abnormalities were not detected, our cases did not appear to be associated with systemic autoimmune disease. Including the present patients, five cases of hepatic RLH accompanying malignancies have been reported ,11,15, sIn summary, we report two cases of RLH of the liver in a patient with colon cancer. RLH of the liver is extremely rare and it is very difficult to think of RLH of the liver as differential diagnosis.RLH: Reactive lymphoid hyperplasia, MALT: Mucosa-associated lymphoid tissue, US: ultrasonography, CT: Computed tomography, MRI: Magnetic resonance imaging, SPIO: Superparamagnetic iron oxideThe author(s) declare that they have no competing interests.HT, MS, YO, and HT carried out the clinical examination and operation. HT, HF, HS, and HI performed pathological analysis. HT and YM participated in the design of the study. TM conceived of the study, and participated in its design and coordination. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Primary malignancies of colorectal origin can metastasise to the bladder. Reports are however extremely rare, particularly from the caecum.The report describes the case of a 45-year old male with Duke's B caecal carcinoma treated with a laparoscopically-assisted right hemicolectomy and adjuvant 5-Fluorouracil chemotherapy. Subsequently, a metastatic lesion to the bladder was demonstrated and successfully excised by partial cystectomy.In order that optimal therapeutic options can be determined, it is important for clinicians to distinguish between primary disease of the bladder and other causes of haematuria. Various immunohistochemical techniques attempt to differentiate primary adenocarcinoma of the bladder from secondary colorectal adenocarcinoma. Suspicion of metastatic disease must be raised when histologically unusual bladder tumours are identified. The vast majority of bladder tumours are primary transitional cell carcinomas. Primary adenocarcinomas of the bladder are rarer but well recognized. Secondary neoplastic growths of the bladder account for approximately 2% of bladder malignancies . BladderA 45-year-old man presented with vague abdominal pain associated with a palpable mass. An ultrasound scan demonstrated a mass in the right iliac fossa but no evidence of hepatic metastases or lymphadenopathy. A plain X-ray film of the chest demonstrated no abnormality. A subsequent barium enema suggested a caecal carcinoma and he was subsequently treated for a pT3 N0 M0 (Dukes B) moderately differentiated mucinous adenocarcinoma of the caecum in July 2002 with a laparoscopically-assisted right hemicolectomy. A complete oncological resection was achieved with clear surgical resection margins of at least 4 centimetres. Fourteen lymph nodes were sampled but no metastatic disease was identified. In concordance with his high risk for microsatellite instability, he underwent 30 sessions of weekly adjuvant 5-Fluorouracil (5-FU) chemotherapy with minimal co-morbidity. Eight months post-operatively he was referred to the Urology Department with a 4-month history of irritative lower urinary tract symptoms that had culminated with a short history of frank haematuria.An initial flexible cystoscopy revealed a solid-looking 2 cm growth on the posterior bladder wall. A subsequent intravenous urogram demonstrated normal upper tracts. He underwent trans-urethral resection of the bladder tumour (TURBT) through to deep muscle. Histology of the TURBT specimen revealed a moderately differentiated adenocarcinoma with the following immunoprofile: CK20 and CDX2 +ve, but CK7 -ve. This tissue contained no evidence of extensive cystitis cystica glandularis, intestinal metaplasia or any in-situ component, as may be seen in a primary bladder adenocarcinoma. Comparison was made with the original caecal pathology. Morphologically and immunohistochemically these specimens were identical, suggesting bladder metastases from the primary caecal malignancy Figure . A compuTen months following the original TURBT, recurrent bladder tumour was found in the same position at check cystoscopy. This was confirmed to be recurrent moderately differentiated intestinal-type adenocarcinoma. Magnetic Resonance scanning revealed a mass involving the right antero-lateral bladder wall and dome extending to the perivesical fat and the right vas deferens. Review of the scans at the Uroradiology multidisciplinary team meeting suggested the lesion to actually be stage pT2b with associated post-TURBT perivesical inflammatory changes. There was no evidence of lymphadenopathy or metastatic disease. Furthermore neither recurrent colon tumour nor any direct involvement between bowel and bladder were identified. Subsequently he was offered and underwent a partial cystectomy Figures . There wDistant metastases to the bladder have been reported in patients with primary stomach ,6, skin A correct distinction between primary adenocarcinoma of the bladder and secondary colorectal adenocarcinoma is important for staging of disease, determining appropriate treatment and ultimate prognosis . In thisAs in this case, immunostains CK7 and CK20 are commonly used to aid diagnosis, with most lesions of colorectal origin having a CK7 negative and CK20 positive profile Figure . The stuThis case highlights the rare complication of a distant bladder metastasis from a caecal carcinoma. This was successfully treated with a surgical resection and the patient had not suffered recurrence after twelve months of follow-up. Suspicion of metastatic disease must be considered when histologically unusual bladder tumours are identified. In the absence of pre-existing malignant disease immunohistochemical panels of antibodies are often required to try and identify the focus of primary disease thus allowing appropriate treatment regimen to be formulated.TURBT: Transurethral resection of bladder tumourThe author(s) declare that they have no competing interests.RJB was the lead clinician responsible for the patient's care, performed the partial cystectomy and along with SBM and RH conceived the idea for the report. RH carried out the pathological and immunohistochemical analysis of the specimens. Furthermore, RH provided appraisal of the pathological discussion. BRG, LC and SBM were also members of the surgical team, reviewed the case notes and drafted the manuscript. All authors read, appraised and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Several malignancies have been described in association with inflammatory bowel diseases, the most common being adenocarcinoma. Carcinoid tumor and Crohn disease has also been previously reported, however the coexistence of both neoplasms is quite rare and the clinical diagnosis is very difficult. Here we report what we believe to be the fourth case of a mixed adenocarcinoid tumor coexisting with Crohn's disease.The patient presented with clinical and radiological features of intestinal obstruction. Laparotomy showed a stricturing lesion in the last 6 cm of the terminal ileum with proximal dilation. Only the histology of the resected surgical specimen proved the presence of a mixed adenocarcinoid tumor involving the terminal ileum.Carcinoid tumor should be suspected in elderly patients with Crohn's disease presenting with intestinal obstruction and laparotomy should be considered to exclude malignancy. It has been recognized that patients with Crohn's disease (CD) are at increased risk of developing malignant lesions ,2. AdenoWe report here a case of ileal Crohn's disease associated with both adenocarcinoma and carcinoid tumor.A 64-year old woman was admitted to the Gastroenterology Unit of another Hospital for abdominal pain, nausea, weight loss, and recurrent episodes of constipation. She had suffered from intermittent pain located in the right lower quadrant for 2 years, but denied chronic diarrhea. She was diagnosed as having Crohn's disease of the terminal ileum by ileocolonoscopy and histology. An upper gastrointestinal series excluded CD in the upper gastrointestinal tract.After inductive therapy with azathioprine and corticosteroids, the patient was maintained with mesalazine 500 mg × 4 daily. Prednisone 50 mg/day was added only during acute phases; in the six years following the discharge, 2 episodes of abdominal pain and dyarrhea requiring steroid therapy were recorded. Seven years later the patient was admitted to our Institution for abdominal pain, nausea, iron deficiency anemia, hypoalbuminemia, and intestinal obstruction. On physical examination no masses in the abdomen were noted. Small bowel double-contrast enteroclysis Fig.  showed aAt operation, there was a stricturing lesion in the last 6 cm of the terminal ileum with proximal dilation. The wall of the affected ileal loop and caecum was thick and fibrous. Resection of the terminal ileum and caecum was performed. The intestinal continuity was obtained with an end-to-end ileocolic hand-sewn anastomosis.The surgical specimen included 28 cm of the terminal ileum and caecum. Grossly, all the length of surgical resection showed diffuse thickness of the intestinal wall. There was no evidence of malignancy Fig. . Only thMalignant tumors of the small intestine are very rare in the general population, so even the few cases reported in the Literature in association with CD are enough to consider CD patients at 4–20 fold increased risk for small bowel malignant neoplasm -5.About 2% of patients affected by CD will develop cancer in the course of their disease, and, in contrast to the patients with ulcerative colitis (UC), those with CD are at risk for developing malignancy even in the first decade of their disease . The coeUntil now, there is no epidemiological evidence that patients with CD are at increased risk for carcinoid tumor . HoweverOnly three cases of a coexistent CD and mixed adenocarcinoid tumor have been reported in the English Literature -10. OursReviewing the Literature ,6,7, we The author(s) declare that they have no competing interests.UC, MDS enrolled the patient for clinical and surgical aspects, drafting the article.SF histological examinations.MMC setting up images, critical revision of the article.AL radiological imaging.ECA performed surgical operations, critical revision of the article, final approval of the versionAll Authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Bancroftian lymphadenovarix was made. On the administration of oral diethylcarbamazine, the diameter of the lymphatic vessels in the lymphadenovarix reduced considerably in size and microfilaremia disappeared. We report this case because axillary lymphadenovarix is a rare presentation of filariasis. This case is also unique since microfilariae were demonstrated in the fluid aspirated from the dilated lymphatics of the lymphadenovarix in the absence of live adult worms.Clinical manifestations of lymphatic filariasis depend on the area of lymphatic involvement and the duration of infection. A 21 year old man, resident in a filariasis endemic region, presented with multiple matted lymph nodes with cystic areas forming a large mass in his left axilla. An ultrasound scan of the axilla using a 7.5 MHz transducer revealed grossly dilated lymphatics but no filarial dance sign. Fine needle (21 G) aspiration cytology (FNAC) from the dilated lymphatics and solid areas in the lymph node mass revealed multiple microfilariae in a background of reactive lymphoid cells. Peripheral blood smears revealed microfilaremia with significant eosinophilia. Diagnosis of left axillary  Lymphatic filariasis is a major health problem in India especially in its southern provinces. A majority of the infected individuals in filariasis endemic communities are asymptomatic. Adult worms in the lymphatics cause progressive lymphatic vascular dilation and dysfunction . ProgresA 21 year old presented in July 2004 complaining of a large painless lump in the left arm pit which he reported as a problem of six months duration. He was a manual laborer working from Tamil Nadu, India. He had no history of episodic fever, cough, weight loss, swelling of the left upper extremity or any lumps elsewhere in the body. Clinical examination revealed a mobile and non-tender mass of matted lymph nodes measuring 5 cm × 5 cm with heterogeneous (soft to firm) consistency with some cystic areas, in the central part of the left axilla  aspiration cytology from the axillary mass revealed a scanty aspirate, smears from which showed features suggestive of reactive lymphoid hyperplasia.Wuchereria bancrofti inside the dilated channels  with a Toshiba (Justvision 400) ultrasound machine  and a 7.5 MHz linear phased array transducer. The scan revealed a lobulated heterogeneous soft tissue mass of size 5.5 cm × 4.5 cm × 5.0 cm. The mass consisted of solid hypoechoic areas (each 2 cm × 1 cm) with multiple dilated tortuous anechoic channels (suggestive of grossly dilated lymphatics) of varying diameter (7 mm to 10 mm). There was no evidence of calcification or the typical filarial dance sign of s Figure . A scrot3. A thick venous blood smear examination taken one hour after the administration of 100 mg oral diethylcarbamazine (in the daytime) (DEC provocation test) revealed numerous typical microfilariae of Wuchereria bancrofti  showed eosinophilia but failed to show any microfilaria. The absolute eosinophil count was 2520/mmi Figure . The micThe patient then received oral diethylcarbamazine (100 mg q8 h) for 3 weeks. In October 2004 the size of the axillary mass and diameter of the lymphatic vessels had reduced in size considerably. This was confirmed by a repeat ultrasound examination of the left axilla (performed by the same sonologist using the same ultrasound machine), which revealed a heterogeneous soft tissue mass of 3.0 cm × 3.0 cm × 4.5 cm with dilated tortuous lymphatic channels, diameter less than 5 mm  in dilated intrascrotal juxtatesticular lymphatics (worm nests) of approximately 80% of microfilaremic but asymptomatic men residing in endemic areas [Lymphatic filariasis is a major health problem in India with most infections caused by 's blood . Ultrasoic areas . In indiic areas . In thesic areas . Microscic areas . Similaric areas . HoweverThe diagnosis of a filarial infection can also been made by detecting microfilariae on microscopic examination of fine needle aspirates from lymph nodes ,8. Fine Our case appears unique for a number of reasons. Firstly, filarial lymphangiectasia presenting as a visible lump (lymphadenovarix) in the axilla is rare. Secondly, the ultrasound scan of the lump did not identify any adult worm(s) in the axilla in spite of significant lymphangiectasia. The absence of worms in the lymphatics on the ultrasound does not, however, rule out their presence in the deeper lymphatics of the axilla. Interestingly, the filarial dance sign has not been detected in the axillary lymphatics of asymptomatic amicrofilaremic individuals residing in a filariasis endemic region of South India . ThirdlyW. bancrofti, axillary lymphatics may also be affected. The host-parasite interaction in these lymphatics may be different from those in the scrotal lymphatics. This may explain the formation of a lymphadenovarix in the axilla and also the dramatic response to diethylcarbamazine in our patient. The authors recommend an ultrasound guided aspiration of fluid from dilated lymphatics of similar lesions in amicrofilaremic patients from filariasis endemic areas.In conclusion, although scrotal lymphatics are the preferred location for adult The author(s) declare that they have no competing interests.AB drafted the manuscript with the help of SCS, SKV and SJ. AB, SCS and SJ conceived and designed the study. AB, SCS and SKV were involved in the acquisition of the data. All authors were involved in analysis and interpretation of the data. All authors read and approved the final manuscript.


The occurrence of skeletal muscle metastases is a very rare event. Only two cases of late skeletal muscle recurrence from cervical cancer have been documented until now.A 38-year old patient, submitted to radical hysterectomy and pelvic lymphadenectomy for a squamous FIGO stage IB1 cervical carcinoma, presented after 76 months with a palpable, and painless swelling on the left hemithorax. MRI showed a nodule located in the context of the intercostal muscles. Pathology revealed the presence of metastasis of squamous cell carcinoma of similar morphology as the primary. On the basis of FDG-PET findings, which excluded other sites of disease, surgical excision of the lesion was performed. The patient was triaged to chemotherapy plus external radiotherapy.A case of skeletal muscle recurrence from cervical cancer after a very long interval from primary diagnosis is reported. Muscular pain or weakness, or just a palpable mass in a patient with a history of cancer has always to raise the suspicion of muscle metastasis. The incidence of skeletal muscle metastases is reported to be less than 1% of metastases of haematogenous origin, despite the fact that skeletal muscle accounts for nearly 50% of the total body weight and is characterized by a rich blood supply [To our knowledge, five cases of skeletal muscle metastasis and two cases of skeletal muscle recurrence after 24 and 60 months fro initial diagnosis of cervical cancer have been documented until now .We report an unusual case of isolated intercostal muscle recurrence after a very long interval from primary diagnosis of squamous cervical cancer. A review of the literature with particular emphasis on the diagnostic and therapeutic issues is also presented.A 38-year old patient was admitted to the Gynaecologic Oncology Unit of the Catholic University of Rome in June 1998 for irregular vaginal bleeding. Gynaecological examination revealed an ulcerated lesion (maximum diameter = 3 cm) of the uterine cervix The histopathologic diagnosis of colposcopy-guided biopsy revealed the presence of moderately differentiated squamous cell cervical carcinoma. The patient was clinically staged as having FIGO stage IB1 cervical cancer and was submitted to radical hysterectomy and pelvic lymphadenectomy. Final staging was pT1bN0M0 according to TNM classification.After 76 months from initial diagnosis, the patient presented with a palpable, painless, mobile and tender swelling on the left side of the thorax approximately at the level of the 5th rib. Ultrasonography was performed revealing a subfascial hypoechogenic lesion (maximum diameter = 2 cm) within the intercostal muscles, close to the rib, which however, appeared uninvolved Figure . MagnetiAfter extensive counselling, the patient was triaged to combined chemotherapy with carboplatin (5AUC) and paclitaxel (175 mg/m2), plus external radiotherapy  on the left hemi thorax. After two months from the completion of treatment, the patient shows no evidence of disease and does not complain of any symptom related to radiation treatment.We first reported a case of isolated intercostal cage muscle recurrence after a very long interval from the diagnosis of squamous cell carcinoma of the uterine cervix. As shown in Table Establishing the diagnosis of skeletal muscle metastasis sometimes can be difficult: indeed, although skeletal muscle metastases can be painful or palpable or cause deformity according to location, sometimes they can be asymptomatic. Indeed, our patient only complained of a mobile, painless swelling, which could have been easily underestimated given the long interval from initial diagnosis, the favourable clinical pathological characteristics of disease , and, above all, the unusual and unexpected recurrence presentation. Although the histopathological evaluation represents a more straight forward diagnostic approach, diagnostic imaging techniques are useful tools in order to define the site and extension of disease , and excIn conclusion, we reported a case of a solitary distant skeletal muscle recurrence from early stage cervical cancer after a very long interval from primary diagnosis. Late recurrence  in cervical cancer patients is considered a very rare event occurring in approximately 4% of cases with a frequency increasing from 1.8% in stage I, to 5.2% and 8.5% in stage II and III disease, respectively . These fThe author(s) declare that they have no competing interests.GF conceived the study, was the coordinator of the study, and drafted the manuscriptVS collected the clinical dataAT was responsible for the clinical surveillanceGFZ was responsible for the evaluation of histopathological dataMP participated in the coordination of the study, and in drafting the manuscriptGS participated in the draft of the study, and in the conception of the studyAll authors read and approved the final manuscriptThe pre-publication history for this paper can be accessed here:


Cystic neoplasms of the pancreas are less common than solid tumors, and portend a better prognosis. They can be divided into serous and mucinous subtypes, with the former behaving less aggressively and generally considered benign. Of the serous neoplasms, serous microcystic adenoma is the most common. An extremely rare solid variant of serous microcystic adenoma lacking secretory capability has been described. Herein, we present the fourth described case of this solid variant and review the literature.We present a case of a 62 year-old man with a history of abdominal pain, who on CT scan was found to have a solid mass at the junction of the head and body of the pancreas. The patient was offered resection for diagnosis and treatment, and subsequently underwent distal pancreatectomy and splenectomy. Based on gross pathology, histology and immunohistochemistry, the mass was determined to be a solid serous microcystic adenoma.Solid serous microcystic adenoma shows similar histologic and immunohistologic features to its classic cystic counterpart, but lacks any secretory functionality. It appears to behave in a benign manner, and as such, surgical resection is curative for patients with this tumor. Furthermore, until more cases of solid SMA are identified to further elucidate its natural history and improve the reliability of preoperative diagnosis, surgical resection of this solid pancreatic tumor should be considered standard therapy in order to exclude malignancy. Tumors of the pancreas generally portend a poor prognosis, the majority of these being highly malignant ductal adenocarcinomas . Much leSerous microcystic cystadenoma (SMA) is the most commonly encountered of the serous cystic neoplasms. On gross examination, SMA typically is a well circumscribed, round to ovoid lesion with a spongy consistency . Often tA rare solid variant of SMA has been described in three previous cases ,10,11. AA 62 year-old man with a several month history of abdominal pain was referred to The Ohio State University Medical Center in June, 2006. His past medical history was significant for hypertension, arthritis and chronic back pain. On computed tomography scan he was found to have a heterogeneously enhancing solid mass at the junction of the head and body of the pancreas Figure . Based oThe patient was offered resection for diagnosis and definitive therapy. After undergoing diagnostic laparoscopy to rule out carcinomatosis in the peritoneal cavity, a distal pancreatectomy with splenectomy was completed.The patient tolerated the procedure well and was discharged after an uneventful hospital stay.Gross examination of the resected specimen demonstrated a 4.2 × 2.5 × 2.0 cm solid, well circumscribed, non-encapsulated mass adjacent to the pancreatic duct Figure . Rare smMucicarmine stain was negative. Immunohistochemistry showed positive staining for cytokeratin 7 (CK7), cytokeratin 19 (CK19), neuron-specific enolase (NSE), α-inhibin and calponin; and negative staining for vimentin, α1-antitrypsin (A1AT), synaptophysin, carcinoembryonic antigen (CEA), renal cell carcinoma stain (RCC), CD10, S-100 protein, and smooth muscle actin. These findings support the final diagnosis of solid microcystic adenoma.For a solid, clear cell tumor in the pancreas, the differential diagnosis includes neuroendocrine tumors, solid pseudopapillary tumor, ductal adenocarcinoma, acinar cell carcinoma, solid serous microcystic adenoma, and metastatic clear cell carcinoma of renal, myoepithelial or other origin. A diagnosis of solid SMA was made in this case after considering gross pathology, histology and immunostaining characteristics.The previous cases of solid SMA were all well-circumscribed, spherical masses ranging in size from 2.5–4.0 cm. Our specimen shared these characteristics and was approximately the same size, with a maximal dimension of 4.2 cm. Histologically our case is virtually identical. All four have had the same general solid architecture: thick fibrous bands separating glandular structures composed of clear, cuboidal cells with central nuclei and abundant glycogen.Further evidence supporting the diagnosis came from immunohistochemical studies. Positive staining for CK7, CK19 and NSE is consistent with the other solid SMA cases, and these markers are generally present in serous microcystic adenomas . The absRenal and myoepithelial cell carcinomas can metastasize to the pancreas, and may be difficult to distinguish from a primary pancreatic cancer . This tuSolid tumors of the pancreas are typically associated with malignancy, whereas cystic tumors more often tend to be benign . Given tThe author(s) declare that they have no competing interests.JRS: data collection, literature review and manuscript preparationWLF: critical reviewECE: critical reviewMB: data collection and critical review


Orbital complications following functional endoscopic sinus surgery (FESS) are fortunately rare. They are usually easily and rapidly recognizable.We present an unusual case of a forty-five year old woman who underwent routine FESS and was not packed nasally after the procedure. Six hours later she started bleeding and nasal packs were inserted. She soon developed unilateral periorbital bruising and within hours her condition had worsened so much that the viability of the eye was thrown into question. She underwent medial and lateral canthotomies and made an uneventful post-operative recovery.This rare case demonstrates that late, brisk post-operative bleeding can occur after FESS with potentially catastrophic consequences. Clinicians should be aware that discharging patients after FESS too early may lead to medico-legal problems. Orbital complications of FESS are fortunately rare . When thLate bleeding causing orbital complications following FESS has been reported before . Post-opManagement of periorbital haematoma depends on the type of haemorrhage  . Slow veDay case surgery has the advantage over in-patient surgery by being cost-effective and resource conserving. It has been promoted by the Department of Health who want 75% of all surgery to be performed in a day case setting by 2010 . HoweverWritten consent was obtained from the patient for publication of study. This case report reflects on a 45 year old woman who underwent routine functional endoscopic sinus surgery (FESS) for recurrent sinusitis. Pre-operatively she was noted to have bilateral concha bellosa on Computerised Tomography (CT) scanning with subsequent obstruction of both osteo-meatal complexes  declare that they have no competing interests.AKA wrote the manuscript, and performed the literature search. HAJ recorded the case history, and contributed to the final manuscript. DM contributed to the literature search and helped write the manuscript.The pre-publication history for this paper can be accessed here:


Metastatic tumours of the stomach present a clinical dilemma for the surgeon. Palliative surgical resection can alleviate symptoms and prolong survival in selected patients. However, previous studies have used open methods of surgical resection with potentially high morbidity and mortality. We describe the use of laparoscopic wedge resection of the stomach for palliative resection of metastatic melanoma to highlight the benefits of this technique.A 58 year old male was investigated for iron deficiency anaemia while under treatment for pulmonary metastatic malignant melanoma. An upper gastrointestinal endoscopy revealed a 5 cm diameter ulcer on the anterior wall of the stomach, biopsies from the ulcer confirmed metastatic melanoma. Laparoscopic wedge resection of the stomach lesion was performed without complication.Laparoscopic approach has many benefits and is useful for the palliative resection of rare tumours of the stomach in order to preserve the quality of life. Its use should be considered in selected patients. Gastrointestinal metastases from malignant melanoma (MM) represent a clinical problem. Previous studies have shown that surgical resection offers good symptom palliation, prevents future emergency presentation and prolongs survival -3. HowevWe describe a patient with a MM deposit in the stomach causing gastrointestinal bleeding and the need for repeated blood transfusion. Treatment was by laparoscopic wedge resection (LWR) using a combined endoscopic and laparoscopic technique. LWR of the stomach is popular technique in Japan for curative resection of early gastric adenocarcinoma and therA 58 years old male was investigated for iron deficiency anaemia while under treatment for lung metastases from melanoma. He had been diagnosed to have cutaneous malignant melanoma 11 years previously. An oesophago-gastro-duodenoscopy (OGD) revealed a 5 cm diameter ulcer on the anterior wall of the stomach  to exclude the tumour Figure . The staThe patient was well when reviewed in outpatients 8 weeks following surgery. There has been no recurrence of his anaemia and further blood transfusions have not been required.Autopsy studies have shown that gastrointestinal metastases are common in patients dying of MM . HoweverThe typical feature of melanoma metastases at gastroscopy is a hemispherical submucosal nodule with central ulceration resembling a "Doughnut" , as in oThe laparoscopic resection of MM metastases to adrenal gland , gallblaPreservation of quality of life is important in the treatment of patients with gastrointestinal metastasis from MM who often have a limited life expectancy. Laparoscopic wedge resection is relatively simple technique and offers advantages of laparoscopic surgery including short hospital stay, early return to normal activity and minimal morbidity and mortality. However, care should be taken in avoiding specimen contact with the laparoscopic port sites as port site recurrence has been reported . The indThe author(s) declare that they have no competing interest.RSD: Obtained patient consent, organised clinical photographs and wrote first draft of the manuscript. EAG: Performed literature search, retrieved articles and helped with manuscript preparation. SAP: Examined surgical specimen, took the photograph of the specimen and helped with manuscript preparation. IMcLW: Edited the manuscript and had overall responsibility for patient care.


The Dabska tumor also known as Endovascular papillary angioendothelioma is a rare type of hemangioendothelioma characterized by intraluminal papillary endothelial structures. Most of these are superficial in location but occurrence in deeper tissues is also known. We describe case report of testicular Dabska tumor in a child presenting as inguinal hernia. To the best of our knowledge this is the first case report describing the occurrence of this rare entity in testis. The Dabska tumor is a rare, low-grade angiosarcoma that often affects the skin and subcutaneous tissues of children . MicroscA 1 year 10 month old male child was referred to our pediatric surgery department for pain and tenderness in a scrotal swelling for past 2 days. The swelling was diagnosed as non-obstructive inguinal hernia at the referral centre at 2 months of age. As it was not progressing the parents were advised to wait till the age of one year. There was no history of any trauma to genitalia. On examination the scrotum was swollen on left side and left testis was not palpable separately. The right testis was normally palpable in scrotal sac. Ultrasonographic examination of the swelling showed a markedly enlarged testis with a heterogenous echotexture. A possibility of testicular tumor was suggested and Fine Needle Aspiration Cytology (FNAC)/biopsy were advised. The FNAC showed few clusters of spindle cells and some cells with prominent nucleoli and vacuolated cytoplasm. However no definite opinion was given. A biopsy was also done which was reported as infarction of testis with extensive vascular proliferation. Subsequently a high left inguinal orchidectomy was carried out. Intraoperatively the hernial sac contained clear fluid and no bowel loops were seen. The specimen was sent for histopathology.The gross examination revealed a tumor replacing the entire testis; however shape of the testicle was maintained figure . The tumThe Dabska tumor was first described in 1969 by Maria Dabska in skin and subcutaneous tissues in children and named as malignant endovascular papillary angioendothelioma. She described 6 cases in her series . Since tAlong with a closely related tumor, the retiform hemangioendothelioma it comes under the category of Hobnail hemangioendothelioma, a subtype of hemangioendothelioma. Pathophysiologcally it has been viewed as a distinct intralymphatic neoplasia and renamed as papillary intralymphatic angioendothelioma . These tOrchidectomy is the treatment of choice. Prognostically these tumors are low grade lesions with a capacity to extend to regional lymph nodes . Three oOur case describes the first occurrence of this entity in testis with an unusual presentation in the form of inguinal hernia. It also highlights the difficulty in diagnosing such tumors on FNAC or small biopsy specimens as it was mistaken for an infarct on biopsy.


Hemangioma is a common soft tissue lesion in the head and neck region. Hemangioma in the context of minor salivary glands is rarely encountered in surgical pathology practice, and for this reason most pathologist are often unfamiliar with its histomorphological features. We report a rare histological finding of salivary gland structures within a cavernous hemangioma, which may or may not have originated in the minor salivary gland. Though hemangioma is a common soft tissue lesion in the oral and maxillofacial region, pathologists are often unfamiliar with the histomorphology of hemangioma in the context of salivary glands , especiaThis is a report of a 55 year old female who presented with pain over the right side of the face for the past 3 years. She stated that the pain evolved spontaneously and is continuous, often disturbing her from sleep. She also stated that she did not get much relief from routine analgesics. Emotionally she was very much depressed, as she had been evaluated by a number of general and oral physicians. But the diagnosis remained elusive. Her attending dental care provider had extracted few teeth, presumably on the assumption that the pain was of dental origin. However, there was no relief and hence she presented to us.On extra oral examination no anomalies were detectable. Intra oral examination showed a brownish red discoloration, measuring 1 × 0.75 cm on the lingual aspect of the right retro molar region. The surface of the lesion was slightly elevated and irregular. There was no pain on palpation but it bleed. The lesion blanched on pressure but immediately refilled upon release. Based on the clinical appearance, a vascular lesion was high on the diagnostic considerations. Panoramic radiograph showed no significant finding. The lesion was excised with proper precautions. During excision the bleeding was little more than normal and continued for 15 minutes, but was arrested following pressure pack application. In the next few days following excision the patient reported that the pain had subsided considerably.Routine microscopy showed predominantly entrapped ducts and effaced acini amid proliferating thin walled endothelium lined cavernous type of blood vessels Fig  &2D. In As in other sites, capillary and cavernous are the two main types of hemangiomas that can occur in salivary glands . CavernoThe diagnosis of hemangioma is often challenging when it involves salivary glands . ComputeThe microscopic features of salivary gland hemangioma is distinctive, and is characterized by effaced acinar structures, retained ducts, and lesional tissue within the confines of salivary gland lobules . AlthougIn the present case, the presence of vascular proliferations in the main substance of the salivary gland associated with retained ducts and effaced acinar units may indicate intraglandular origin, as it is at a deeper location than one would expect. But in the absence of any specific histological parameters to assess minor salivary gland origin with certainty, it is virtually impossible to exclude secondary involvement .The comparison of the present case with arteriovenous hemangioma merits attention because of the presence of pain, which is a prominent symptom in arteriovenous hemangioma  and is pIn conclusion, the present lesion shows definite involvement of minor salivary gland by a hemangioma, a rare histological finding, which may or may not have originated in the gland.


Granular cell tumors (GCTs) are extremely rare lesions of the urinary bladder with only nine cases being reported in world literature of which one was malignant. Generally believed to be of neural origin based on histochemical, immunohistochemical, and ultrastructural studies; they mostly follow a clinically benign course but are commonly mistaken for malignant tumors since they are solid looking, ulcerated tumors with ill-defined margins.We herein report two cases of GCTs, one benign and one malignant, presenting with gross hematuria in a 14- and a 47-year-old female, respectively.Histopathology revealed characteristic GCTs with positive immunostaining for neural marker (S-100) and negative immunostaining for epithelial , neuroendocrine  and sarcoma  markers. The benign tumor was successfully managed conservatively with transurethral resection alone while for the malignant tumor, radical cystectomy, hysterectomy with bilateral salpingo-oophorectomy, anterior vaginectomy, plus lymph node dissection was done. Both cases show long-term disease free survival.We recommend careful pathologic assessment for establishing the appropriate diagnosis and either a conservative or aggressive surgical treatment for benign or localized malignant GCT of the urinary bladder, respectively. Granular cell tumors are unusual, rare neoplasm that most commonly affect the head and neck region, especially the tongue. Only nine cases of granular cell tumors of the urinary bladder have thus far been reported. Although usually benign, these lesions may present as solid tumors with ill-defined margins and ulcerated surface, masquerading as a malignant tumor at initial presentation, and hence could be confused with transitional cell/squamous cell carcinoma or sarcoma in the urinary bladder. Only one case of malignant granular cell tumor of the urinary bladder has thus far been reported. We herein, report two cases of granular cell tumor of the urinary bladder, one benign and one malignant, and review the literature with a view to comment on the existing experience about the presentation, diagnosis and management of this rare bladder tumor.per urethram. Heart rate was 120/minute, blood pressure 90/55 mmHg and she was afebrile. Hemoglobin was 5.9 gm/dl, hematocrit 18 with coagulation profile and routine chemistry being normal. Urinalysis showed large amounts of RBC's and 08 WBC's/HPF. Urine culture and sensitivity was later negative for bacterial growth. At emergency cystoscopy following resuscitation, she was found to have an 8 × 10 cm highly vascular, solid, infiltrative tumor occupying the right lateral wall and ipsilateral half of the trigone. The tumor involved the right ureteric orifice and extended to 1 cm short of the bladder neck. Preoperative intravenous pyelogram showed a non-excretory right kidney due to hydronephrosis with normal contralateral system. Transurethral resection of the tumor till bladder wall level was done. She needed 5 units of packed cells transfusion. Subsequently, right percutaneous nephrostomy was done, which was later internalized with a double J stent.A 14-year-old girl presented to the Emergency Room in December 1999 with her first episode of severe gross, painless hematuria. Past history was unremarkable. On examination she was pale, dehydrated and continually bleeding Microscopic examination of the tissue revealed cohesive groups of cells arranged in lobules by dividing fibrous septae. There was no evidence of muscle invasion. The cells contained abundant granular eosinophilic cytoplasm with round monomorphic nuclei and showed focal positivity with PAS stain Figure  &1b. ImmCT scan of the abdomen/pelvis and chest X-ray showed no metastases. There was localized thickening of the bladder wall with no extravesical extension. At repeat cystoscopy 2 weeks later, there was no obvious residual tumor and deep biopsies from previous resection site were clear. Hence, it was decided to treat the patient conservatively. Follow-up CT scan of the abdomen/pelvis 4 months later showed a normal-looking bladder  with coagulation profile and routine chemistry being normal. Urinanalysis showed hematuria and pyuria while urine culture was negative for bacterial growth. Ultrasound revealed a 2.6 × 1.8 cm polypoidal mass in the left posterior wall of urinary bladder with bladder wall irregularity. An IVP showed normal upper urinary tracts with a filling defect in the bladder.Cystoscopy revealed an approximately 4 × 3 × 3 cm solid tumor in the left postero-lateral wall, above the ureteric orifice. Multiple biopsies of the tumor from the base and edges, as well as random bladder biopsies, were obtained.The tumor was arranged in large clusters with diffuse sheets invading and insinuating in between the smooth muscle fibers. The tumor cells exhibited pleomorphism, nuclear hyperchromasia, with 2–3 mitoses/10 HPF Figure  &3b. RanA radionuclide bone scan was negative for metastasis. A CT scan of abdomen and pelvis showed no extravesical tumor extension or distant metastasis. A cystic left ovarian mass was recognized, which later turned out to be benign ovarian cyst with no malignancy. A planned radical cystectomy, bilateral salpingo-oophorectomy, hysterectomy, ileal conduit and pelvic lymph node clearance with resection of the anterior wall of the vagina was carried out in June 1995 realizing the malignant nature of the tumor. She made an uneventful recovery and histopathology confirmed a pT3N0M0 malignant granular cell tumor of the urinary bladder. She remains free of disease recurrence at 8 years since the operation.Granular cell tumor (GCT) is an extremely rare neoplasm affecting the urinary bladder with only 9 cases described so far Table  1-9]. A-9. A1-9]The entity was originally described by Abrikosoff  in 1926 Histologically, Granular cell tumor of the urinary bladder is indistinguishable from similar tumors occurring at other sites. Immunohistochemical studies are particularly useful to differentiate such tumors from carcinomas and sarcomas as GCTs reveal positive staining for S-100 protein, calretinin, alpha subunit of inhibin HLA-DR, laminin and various myelin proteins . The celGranular cells are not unique to granular cell tumors as cytoplasmic granularity typical of GCT  has been observed in neoplastic and in non-neoplastic conditions, such as ameliobiastoma , ameliobGCT is rarely diagnosed prior to microscopic examination of the biopsy or excised specimen and often is an incidental finding during routine physical examination. As a rule, GCTs generally tend to follow a benign clinical course and of the nine previously reported cases of GCT of the urinary bladder, only one was malignant Table . ConservMalignant GCT although well established is extremely rare and poses a difficult diagnostic problem. Benign and malignant GCT could be similar in histological appearance. Clinical features such as rapid growth, presence of metastases and local recurrence generally indicates the tumor to be malignant. Microscopic features that favor a diagnosis of malignancy include cellular growth in sheets and clusters with invasion, nuclear hyperchromasia, presence of necrosis, and pleomorphism. Malignant GCTs tend to be slightly more cellular with smaller cells assuming spindle cell morphology. It has been noted that cellular variability or pleomorphism alone is not always a reliable diagnostic criterion. Other features especially when seen in combination such as necrosis, large vesicular nuclei with large nucleoli and high MIB-1 values favor malignancy. There is also an appreciable mitotic rate, although not apparent microscopically, 2 or more mitotic figures10/HPF should raise the suspicion of malignancy ,17.Our second case had features and histologic evidence of malignancy. We therefore opted for radical cystectomy. The patient remains free of disease recurrence 8 years since surgery and we recommend radical cystectomy as the standard therapy for invasive malignant GCT with no metastasis.Granular cell tumors (GCTs) are extremely rare lesions of the urinary bladder. They mostly follow a clinically benign course but are commonly mistaken for malignant tumors since they are solid looking, ulcerated tumors with ill-defined margins. We recommend careful pathologic assessment for establishing the appropriate diagnosis and either a conservative or aggressive surgical treatment for benign or localized malignant GCT of the urinary bladder, respectively.The author(s) declare that they have no competing interests.FA: Primary author and consultant urologistAM: Contributing author and consultant urologistTS: Research officer and performed literature reviewNK: Consultant pathologist who performed pathological assessment of the case, contributing authorNAA: Fellow Urology, involved in data collection, pathological assessment and report preparationAll authors read and approved final version of the manuscript for publication


Malignant peripheral nerve sheath tumors are rare tumor entities that originate from peripheral nerve sheaths and have an unfavorable prognosis. Metastatic spread to the cerebral parenchyma is absolutely rare. This case report describes the clinical course in a 60-year-old man whose tumor came to medical attention because of a seizure.Magnetic resonance imaging demonstrated two intracerebral lesions. The symptomatic lesion was removed microneurosurgically and histology demonstrated a metastasis from a malignant peripheral nerve sheath tumor. Postoperatively, whole-brain irradiation was performed. The primary tumor was identified in the area of the sciatic nerve on the right. Follow-up 14 months after resection showed that there was no progression of the intracerebral lesions but an increase in size and number of distant metastases.There are no generally accepted guidelines for the treatment of malignant peripheral nerve sheath tumors with cerebral metastases. This case report presents and discusses one possible therapeutic approach. Due to the poor overall prognosis, the least invasive therapy should be chosen. The term "malignant peripheral nerve sheath tumor"(MPNST) comprises various entities including malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma. MPNSTs are rare with an incidence of 1:100,000. About half of the tumors occur in patients with type I neurofibromatosis. InteresA 60-year-old man with mental retardation of unclear origin who was otherwise healthy suffered a focal seizure with speech disturbance. Two intracerebral lesions were identified by magnetic resonance imaging (MRI) of the head Figure .The patient's neurological status on admission was normal. There were no clues to phakomatosis in the patient's or in the family history. Neither did the clinical findings point in this direction. No further seizures occurred after initiation and starting phenytoin therapy.Initial MRI demonstrated a contrast-enhancing lesion in the left frontal lobe and a smaller lesion in the right frontal lobe Figure . No otheSince the lesions had no mass effect, initially a stereotactic biopsy was obtained from the left frontal lesion. Histologically, a semimalignant melanocytoma with the differential diagnosis of a MPNST was diagnosed. The lesion was then removed microneurosurgically. Postoperatively, the patient underwent whole-brain irradiation (30Gy). Due to the advanced stage of the malignancy and to gain control over further intracerebral metastasis, which were not visible on the MRI, no local radiotherapy such as radiosurgery was chosen. No postoperative or radiation-related complications were observed.Fluorodeoxyglycose positron emission tomography (FDG PET) identified no body lesions except for an area of increased accumulation lateral to the right jugular vein, which corresponded to an enlarged lymph node, suggesting a nodal metastasis on MRI. PET even failed to demonstrate the right frontal lesion that was still present prior to cerebral irradiation. Only whole-body MRI T2WI) identified several structures of increased signal intensity in the right thigh. One of the lesions was contiguous with the sciatic nerve  staining demonstrated tumor tissue with spindled epithelioid cells, some of which contained melanin Figure . The celThe diagnosis of a MPNST is a clinical challenge for the physician regarding treatment options and additional diagnostic steps. In particular, if the tumor has spread into the Central Nervous System. No uniform criteria have been established for the treatment of MPNST outside the central nervous system. However, it is widely agreed that the extent of surgical resection is an important prognostic factor while no definitive data is available on the benefit of postoperative radiotherapy. IrradiaIn most published cases of MPNST metastasizing to the cerebral parenchyma, the metastasis was removed microsurgically. The majority of reports are from Japan Table . In someMPNST in general has an unfavorable prognosis with a 5-year survival rate of only 64%. Survival is even much shorter in patients with a brain metastasis and is not affected by the therapeutic approach. A definitive appraisal of the situation is difficult because only few patients have been treated so far. One patient survived for 18 months without treatment . In our Beside the clinical challenge of a new diagnose MPNST, the pathologist has to deal with a great work up in the beginning to provide the physician with a correct diagnosis. The histological diagnosis of MPNST requires careful differentiation from a number of tumor entities with similar histologic features. In general, MPNST cells have either an epithelioid or spindled architecture with gradual transitions between the two. The spindled type must be carefully differentiated from other spindled-cell soft tissue tumors, in particular fibrosarcoma. An immunoreaction to S-100 as in our case excludes a fibrosarcoma and underlines the close association of MPNST with the nerve sheaths,10. AnotThe author(s) declare that they have no competing interests.J.T.: collected clinical data, wrote particular parts of the paper, performed the reference searchK.M.: histological evaluation, wrote the histology partG.N.: performed all MRI's, included the figures, wrote the MRI findingsJ.L.: responsible for the irradiation of the patient, contribute in the follow-up of the patient and in the revision of the manuscriptJ.V.: contributed in data collection, revision and translation of the manuscript, performed surgeryThe pre-publication history for this paper can be accessed here: