Choroid plexus papilloma diagnosed by crush cytology

In a 32-yr-old man, an infratentorial cystic lesion with a mural nodule was interpreted to be either a hemangioblastoma or a cystic astrocytoma on CT scan. Intraoperative crush cytology revealed it to be a choroid plexus papilloma (CPP). The utility of crush cytology in the rapid diagnosis of central nervous system (CNS) tumors and the differential diagnosis of CNS papillary lesions are highlighted in this report.     

Malignant transformation of ovarian mature cystic teratoma with a predominant pulmonary type small cell carcinoma component

A 68-year-old woman was diagnosed with mature cystic teratoma of the left ovary when she was 44 years old. The tumor recently enlarged rapidly, and abdominal magnetic resonance imaging revealed an intrapelvic cystic lesion, which measured 123 × 120 × 107 mm and contained a mural nodule. Under a clinical diagnosis of malignant transformation of mature cystic teratoma, bilateral salpingo-oophorectomy with total hysterectomy and omentectomy were performed. The resected specimen showed a unilocular cystic lesion containing a well-demarcated mural nodule measuring 35 × 30 × 25 mm in the left ovary. A microscopic examination revealed various types of carcinoma in the mural nodule: pulmonary type small cell carcinoma (65%), adenocarcinoma (25%), squamous cell carcinoma (5%), and transitional cell carcinoma (5%). Small cell carcinoma was positive for CD56, synaptophysin, and chromogranin A. The adenocarcinoma component showed intestinal phenotypes; i.e. cytokeratin (CK) 7(-), CK20(+), CDX2(+), estrogen receptor(-), and progesterone receptor (-). Interestingly, CDX2 positivity was retained in all of the carcinomas. It was assumed that the adenocarcinoma had arisen from an intestinal epithelium in the mature cystic teratoma and then differentiated into the diverse histological types mentioned above.     

血管母细胞瘤40例临床病理分析

目的探讨血管母细胞瘤临床病理学特征、影像学特点、免疫表型、诊断及鉴别诊断。方法应用HE染色和免疫组化标记等方法对40例血管母细胞瘤进行病理学特征观察,同时分析其CT和MRI影像学特点。结果40例血管母细胞瘤临床表现主要为头晕、头痛、呕吐、视乳头水肿、共济失调等。CT和MRI检查示颅内界限尚清的囊实性病变,内有壁结节,增强扫描后囊性部分无强化,附壁结节明显强化。组织学检查肿瘤主要由两种成分构成:即不同成熟阶段的毛细血管和毛细血管网之间丰富的间质细胞。免疫组化肿瘤组织中的血管内皮细胞均表达CD34和FⅧRAg,间质细胞大多表达S-100,少数表达NSE,血管内皮细胞和间质细胞均表达vimentin,而一般不表达GFAP、EMA和p53,Ki-67表达较低或不表达。结论血管母细胞瘤主要由不同成熟阶段的毛细血管和来源未定的肿瘤性间质细胞构成。其影像学表现具有一定的特异性。血管母细胞瘤须注意与毛细胞型星形细胞瘤、血管瘤型脑膜瘤、转移性肾癌等鉴别。     

August 2002: 21-year-old male with cystic intracerebral tumor

The August 2002 COM. A 21-year-old male presented with a single episode of generalized tonic clonic seizures. Radiology revealed a cystic tumor with mural nodule suggestive of a pilocytic astrocytoma. However, histopathological examination and electron microscopy revealed features of an intracerebral schwannoma. Therefore, although rare, in an intracerebral cystic lesion with mural nodule, the possibility of an intracerebral schwannoma should be entertained. This is important because this is a benign tumor with favourable response to resection.     

Ovarian mucinous cystadenocarcinoma with mural nodule of anaplastic carcinoma and synchronous cervical squamous carcinoma

Solid mural nodule within a mucinous cystic ovarian tumor occurs more often than generally presumed. One especially interesting case involving coincidental cervical carcinoma is presented. A 38-year-old woman underwent exploratory laparotomy for a right ovarian tumor. After ovarian malignancy had been diagnosed from frozen section, the bilateral salpingo-oophorectomy and hysterectomy was performed. The tumor had a unilocular cystic cavity and a mural nodule. The nodule showed undifferentiated carcinomatous features. The immunohistochemical examination revealed atypical cells in the nodule which were positive for cytokeratin, CEA, and vimentine, establishing its anaplastic nature. A synchronous cervical invasive squamous carcinoma was documented. The patient was treated with chemotherapy and radiotherapy. Currently, at 15 postoperative months, she is well and free of disease. The occurrence of ovarian mucinous cystadenocarcinoma with mural nodule of anaplastic carcinoma and cervical squamous cell carcinoma is evidently very uncommon, because we have not found a similar case in the literature.     

Mammary carcinoma with prominent cytoplasmic lipofuscin granules mimicking melanocytic differentiation

We report a case of mammary carcinoma with striking cytoplasmic pigmentation in a 60-year-old Japanese woman who presented with a self-evident nodular lesion in the left breast. METHODS AND RESULTS: After a fine needle aspiration revealing atypical clusters of cells, an excisional biopsy was performed. Histologically, a partially cystic 18 mm lesion containing a 5-mm mural nodule was present. The mural nodule and adjacent thickened epithelium were comprised of atypical cells focally invading into the cyst wall. Striking abundant granular brown pigment resembling melanin was present in some of the neoplastic cells. The differential diagnosis included metastatic melanoma and mammary carcinoma with melanocytic differentiation. After a series of special stains and immunohistochemical studies, the diagnosis of mammary carcinoma with extensive cytoplasmic lipofuscin pigment was rendered. CONCLUSION: Mammary carcinoma with lipofuscin pigment to the degree seen in this case which mimics melanocytic differentiation has not, to our knowledge, previously been documented.     

Fine needle aspiration biopsy diagnosis of primary clear cell chondrosarcoma: A case report

Clear cell chondrosarcoma is a rare chondrosarcoma variant often involving the long bone epiphyses of young to middle aged adults. We report herein a case involving the left femoral head in a 25‐year‐old female with a 3‐month history of worsening left hip pain. Radiographs revealed a complex, multifocal and lytic lesion centered in the left proximal femoral epiphysis with involvement of the femoral neck. Computed tomography‐guided fine needle aspiration biopsy with concomitant core needle biopsy was performed, and a diagnosis of clear cell chondrosarcoma was rendered. Cytologic smears revealed aggregates of matrix material accompanied by a population of mostly uniform spindled to epithelioid and histiocytoid cells, rarely accompanied by osteoclast‐type giant cells. The patient underwent surgical resection with ‐total hip replacement, and subsequent pathologic examination confirmed the initial needle biopsy diagnosis. There has been no evidence of local recurrence or distant metastases with 3‐years follow‐up. To our knowledge, this is the first reported example of a primary clear cell chondrosarcoma initially evaluated by fine needle aspiration biopsy.     

Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature

Central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.     

小脑血管母细胞瘤的MR诊断

目的探讨血管母细胞瘤的MRI诊断。方法回顾性分析经手术病理证实的24例小脑血管母细胞瘤，探讨其MRI表现。结果18例血管母细胞瘤位于小脑半球，6例位于小脑蚓部。15例表现为大囊小结节型，结节强化明显；9例表现为实质型；24例瘤内或瘤周均可见血管流空信号。本组MRI正确诊断22例，准确率为91．67％。结论MRI是诊断小脑血管母细胞瘤的有效检查方法，但不典型者需与胶质瘤、转移瘤、动静脉畸形等鉴别。     

47‐Year‐Old Man with Left Leg Numbness

A 47‐year‐old white male with a history of uveitis, hypercalcemia and nephrolithiasis presented with acute onset partial seizure. On exam he had decreased sensation to light touch on his left lower extremity. A Brain MRI revealed a right frontal mass, which was initially thought to be a metastatic lesion or a primary brain tumor. However, biopsy of the lesion revealed it to be a non‐caseating granulomatous lesion consistent with neurosarcoidosis.     

Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor

The authors described a case of a patient with co-existing endolymphatic sac tumor (ELST) and hemangioblastoma in the posterior cranial fossa, which belonged to a subtype of Von Hippel-Lindau (VHL) disease confirmed by the test of VHL-gene. The signs in this 42-year-old female included intermittent headache and dizziness. Imaging revealed a giant mass in the right cerebellopontine angle (CPA) region and another lesion in the left cerebellar hemisphere. The results of biopsy after two operations confirmed the diagnosis respectively. Both of the tumors were resected totally. Nevertheless, we had to confess the misdiagnosis as vascular tumor instead of ELST at the initial diagnosis because of the rarity of ELST associated with atypical histological characteristics. The purposes we reported this case were to describe the atypical pathological feature of ELST and the mutation of germline VHL not mentioned in previously literature, furthermore, to foster understanding of ELSTs with the avoidance of the similar misdiagnosis as far as possible in future.     

Mucinous tumor of the gallbladder with a separate nodule of anaplastic carcinoma

A case of mucinous tumor of the gallbladder with a separate nodule of anaplastic carcinoma is reported. The patient was an 83-year-old Japanese man who underwent cholecystectomy under the preoperative diagnosis of a mucus-producing gallbladder tumor. A mucinous tumor was found in the neck and distal body of the gallbladder, associated with a separate nodule in the fundus. The latter nodule was initially diagnosed as a benign xanthogranulomatous lesion. However, the immunohistochemical study revealed that the atypical cells in the superficial part of the nodule were positive for cytokeratin and epithelial membrane antigen, confirming the diagnosis of anaplastic carcinoma. Although the occurrence of mural nodules in mucinous cystic tumors of the ovary and pancreas is well reported, to our knowledge, this is the first report on the occurrence of a mucinous tumor with a nodule of anaplastic carcinoma in the gallbladder.     

Successful treatment of left atrial auricular abscess

Mural endocarditis causing myocardial abscess without valvular involvement is very rare. We report an unusual case of left atrial auricular abscess which was successfully treated by surgical resection, treatment with antibiotics, and mediastinal irrigation. A 9-yr-old female patient with previous history of urinary tract infection was admitted because of persistent fever. Echocardiography and magnetic resonance imaging revealed massive pericardial effusion and a mass lesion at the left upper cardiac border. Pericardiocentesis isolated Staphylococcus aureus on culture. The patient underwent mass removal under cardiopulmonary bypass. The mass was located in the left atrial auricle with fibropurulent abscess formation inside. Postoperative mediastinal irrigation was performed using povidone iodine solution. Pathological examination of the mass showed organized thrombi with chronic fibrosing mural endocarditis.     

Melanin pigmented oncocytic metaplasia of the nasopharynx

 A 64-year-old man presented with a history of discomfort of the throat of a few weeks’ duration. Nasoscopic examination revealed multiple small, brown pigmentations at the left suprapharynx, the base of the left nasal cavity and the pharyngeal openings of the auditory tube on both sides. Microscopically, the lesion showed a glandular pattern of oncocytic epithelium with abundant pigmented granules and melanophages in the surrounding stroma. Immunohistochemically, the dendritic cells in the basal layer were positive for S-100 protein. Electron microscopic study revealed numerous fully melanized melanosomes and hypertrophied mitochondria in the oncocytic cells. Oncocytic cells do not produce melanin for themselves, melanin granules apparently being transferred from the adjacent dendritic cells to the oncocytic cells. Received: 25 August 1998 / Accepted: 13 January 1999     

Phaeohyphomycotic cyst caused by a recently described species, Phaeoannellomyces elegans.

An 81-year-old man presented with a chronic, painful nodule on the palmar surface of the left fourth finger. As a former farm worker, the patient acknowledged frequent soil-contaminated wounds of the left hand 4 to 12 years previously, but he denied any recent trauma. The patient's other medical problems included a history of chronic immunoglobulin A gammopathy and a new pleural mass eroding into adjacent ribs on chest X-ray. The finger nodule was excised and consisted of an intact phaeohyphomycotic cyst which yielded growth of a darkly pigmented fungus. At 25 degrees C, the isolate formed annellidic yeast cells having dark-brown walls consistent with the recently described species Phaeoannellomyces elegans. In vitro antifungal susceptibility tests indicated resistance to amphotericin B and variable susceptibility to imidazoles. The lesion was excised, and the patient received no antifungal therapy. After 9 months of follow-up, the fungal infection shows no signs of recurrence.     

IgG4-related disease manifesting as sclerosing orbital inflammation and cutaneous pseudolymphoma with crystal-storing histiocytosis

A 69-year-old man presented with swelling of the left lower eyelid, proptosis and a history of erythematous scalp nodules. A CT scan revealed a mass involving the left inferior orbit and both lacrimal glands. Laboratory tests revealed peripheral blood eosinophilia and an elevated concentration of serum IgG. Orbital biopsy showed a fibro-inflammatory lesion with plasma cells and eosinophils. The largest scalp nodule was a fibro-inflammatory mass containing lymphoid follicles, plasma cells and eosinophils, as well as CD68-positive cells with eosinophilic, granular cytoplasm. In both lesions plasma cells were polyclonal and expressed IgG and IgG4. Treatment with prednisone and azathioprine resulted in a dramatic reduction in orbital swelling and resolution of the scalp nodules. After 8 months treatment was changed to tamoxifen with no recurrence of orbital disease but intermittent small scalp lesions. Sclerosing orbital inflammation and cutaneous pseudolymphoma have been recognized as IgG4-related diseases. Crystal-storing histiocytosis has been described in inflammatory and neoplastic proliferations of plasmacytoid cells but not in the setting of IgG4-RD. Tamoxifen has been used to treat idiopathic retroperitoneal fibrosis and may be useful for other lesions of IgG4-RD.     

Congenital arteriovenous malformation associated with progressive hydrocephalus in a newborn.

Intracranial arteriovenous malformation is rarely presented in newborns or infants. We describe an unusual case of congenital arteriovenous malformation of the brain with multiple sequestered grape-like venous sacs presented with congenital hydrocephalus. This 4-month-old girl born with a large head, presented with progressive hydrocephalus over a period of 4 months. The brain CT showed multiloculated cysts with a high-density mural nodule and thin cerebral mantle. The right lateral ventricle was collapsed by the cystic lesion, and the contralateral ventricle was markedly dilated, which was thought to be due to aqueductal obstruction by the conglomerated nidus of the arteriovenous malformation. Surgical removal of both hemispheric masses, including the overlying thin mantle. The lesion was pathologically confirmed as the arteriovenous malformation which was composed of markedly dilated veins and multiple sequestered aneurysmal sacs. The overlying cerebral tissue was dysplastic and partly infarcted. This case shows that intracerebral arteriovenous malformation is indeed a congenital anomaly and suggests that intrauterine vascular compromise can result in focal or wide maldevelopment of the brain.     

Intradural extramedullary leptomeningeal hemangioblastomatosis and paraneoplastic limbic encephalitis diagnosed at autopsy: an unlikely pair

Typical cerebellar or spinal cord hemangioblastoma is often solitary and has a benign clinical course, whereas disseminated hemangioblastomatosis is extremely rare. We report a 75-year-old man with disseminated "leptomeningeal" hemangioblastomatosis and concurrent paraneoplastic limbic encephalitis who died of this disease. The patient presented with gait problems and cognitive deficits, and was diagnosed with a cervical spinal tumor. Surgical resection achieved an apparent gross total resection, but later the patient began experiencing severe cognitive decline. His condition progressively deteriorated during the next few months, and he died 15 months after his initial surgery. Autopsy revealed disseminated extramedullary intradural hemangioblastomatosis with extensive leptomeningeal nodules involving the entire spinal cord, medulla, pons, and midbrain, as well as histologic features of limbic encephalitis. There was no evidence of von Hippel-Lindau disease, and no specific etiology was identified for the dementia. To the best of our knowledge, this is the first case of limbic encephalitis occurring in the setting of disseminated hemangioblastomatosis.     

Primary retroperitoneal mucinous cystadenoma with sarcoma-like mural nodule

Primary retroperitoneal cystadenomas are extremely rare. This is the first report in literature to describe a primary retroperitoneal cystadenoma with a sarcoma-like mural nodule. A 45-year-old woman complained of a left-sided abdominal mass. A computed tomography scan revealed a cystic mass with a mural nodule, which seemed to originate from the tail of the pancreas. At laparotomy the cyst was not adhered to the pancreas but localized retroperitoneally. Histologic examination showed a mucinous cystadenoma with only foci of borderline malignancy with a mural “sarcoma-like” nodule. In view of the surgical and histopathological findings, the mucinous cystadenoma was regarded as primary retroperitoneal. This case demonstrates that in the era of radiological preoperative refinement, pathological diagnosis remains of utmost importance, especially for rare cases.     

A case of vasculitis syndrome associated with epididymitis]

A case of vasculitis syndrome associated with epididymitis is reported. A 56-year-old male presented with the sudden left testicular pain and fever. He came to the department of urology in our hospital, and was treated with antibiotics and anti-tuberculous drugs. However, the testicular pain was not relieved as well as the fever. In January 5, 1993, he was admitted. He had lost 6 kg. Physical examination revealed pyrexia and blood pressure of 150/91 mmHg. Laboratory finding revealed leukocytosis, thrombocytosis, and erythrocyte sedimentation rate of 100 mm/hr. On serological examination, the C-reactive protein was positive. We suspected this case as a polyarteritis nodosa, then he was given prednisolone. On January 20, 1993, left orchidectomy and spermatic cord biopsy was performed and non-necrotizing vasculitis was found. On August 10, cyclophoaphamide was added and his symptom disappeared. Although polyarteritis nodosa is well known as the vasculitis affecting the epididymis, it was rare that initial manifestation was testicular pain. In this case necrotizing vasculitis was not proven, but we diagnosed this case as a vasculitis in which the initial symptom was the epididymitis.