Thrombotic thrombocytopenic purpura and cardiac papillary fibroelastoma: a 'unique coexistence'.

Thrombotic thrombocytopenic purpura (TTP), a complex thrombotic microangiopathy, remains an evolving enigma. A 49-year-old African-American woman presented with acute left hemiplegia, an ischemic cerebrovascular accident involving the right middle cerebral artery. Sequential appearance of thrombocytopenia and evidence of microangiopathic haemolysis led to the diagnosis of acquired idiopathic autoimmune TTP. This was managed with plasma exchange (PEX) and steroids. Early haematologic relapse within a month was managed with the addition of rituximab attaining sustained remission. The patient presented 3 years later with acute confusion and expressive aphasia due to multiple infarcts involving the left parieto-occipital cortex. Transoesophageal echocardiography demonstrated a pedunculated 6 mm mitral valvular mass consistent with a papillary fibroelastoma. Anticoagulation was instituted and the patient was continued on therapeutic oral warfarin. A haematologic relapse of TTP eventually emerged and was managed with PEX, steroids and rituximab. This vignette demonstrates several dilemmas in the clinical presentation, diagnosis and management of TTP in current day practice. Rituximab has adjuvant benefits to PEX and is being investigated as potential first-line therapy. Monitoring ADAMTS13 activity and inhibitor titre, as in our case, prove to have prognostic significance. Cardiac fibroelastomas are rare benign cardiac tumours usually arising from valvular endocardium with thromboembolic potential. One of the proposed mechanisms of origin of these masses is organizing thrombi in the setting of endocardial injury and inflammation questioning a possible link to thrombotic microangiopathy. To the best of our knowledge, this is the first report of this unique coexistence.

Carcinoma de células fusiformes tipo “fibromatosis-like”: una variante inusual de carcinoma metaplásico de mama asociado a carcinoma ductal / No disponible

El carcinoma de células fusiformes tipo fibromatosis-like esuna variante del carcinoma metaplásico, que ha sido reconocidorecientemente como una entidad distinta e independiente del restode los tumores metaplásicos, que recuerda a la fibromatosis yse caracteriza por ser un tumor de bajo grado, con mejor pronósticoy tendencia a la recidiva local. Presentamos un caso de unapaciente de 71 años con un carcinoma tipo fibromatosis-likeasociado a un carcinoma ductal de la mama. La histología revelóun tumor de células fusiformes simulando una fibromatosis, dondese puede identificar un componente epitelial en forma de carcinomaductal o intraductal en continuidad con el componente deaspecto fusiforme. Inmunohistoquímicamente presentó positividadfocal para marcadores epiteliales y mioepiteliales como citoqueratinasy expresión de marcadores mesenquimales como vimentina.El diagnóstico exacto puede presentar dificultad tantoradiológica como anatomopatológica y plantea el diagnóstico diferencialcon lesiones benignas como fibromatosis, fascitis nodularo malignas como sarcomas. El comportamiento y pronósticono ha sido del todo aclarado aunque se ha visto que es un tumorque se caracteriza por un alto riesgo de recidiva, bajo potencialpara metastatizar en ganglios linfáticos regionales pero con capacidadpara producir metástasis a distancia y por tanto, debería sertratado en consecuencia(AU)

Fibromatosis-like spindle cell carcinoma of the breast is avariant of metaplastic carcinoma that has recently been recognizedas a different entity because of its resemblance to fibromatosisand similar propensity for local recurrence. We presenta case of 71- year-old lady with a fibromatosis-like carcinomaassociated with ductal carcinoma of the breast. Finalhistology revealed a tumor with predominant spindle cells in acollagenous background, simulating a fibromatosis. Inmunohistochemistryshowed focal positivity of ephithelial and myoephitelialmarkers as citokeratins and expression of mesenchymalmarker as vimentin in the tumor. This tumor can posediagnostic difficulty radiologic as histopathology and the differentialdiagnosis includes both benign and malignant spindlecell breast lesions as a fibromatosis, nodule fascitis or sarcomas.The behaviour and prognosis have not been well clarifiedalthough there seems to have high risk of local recurrence, lowpotential to metastasize to regional lymph nodes and potentialfor distant metastasis and should be treated accordingly(AU)

Left ventricular cardiac fibroma in a child presenting with ventricular tachycardia.

Cardiac tumors in children are rare. Although most are histologically benign, they can be associated with life-threatening arrhythmias and sudden death. We report a 7-year-old boy, with a first episode of symptomatic tachycardia, who was found to have a left ventricular (LV) fibroma. He had a normal echocardiogram prior to an electrophysiology study, which revealed a sustained monomorphic ventricular tachycardia and a radio-opacity near the LV apex. These findings prompted a cardiac MRI, which demonstrated a discrete mass on his LV apex and free wall. Our case emphasizes that structural heart disease should be aggressively pursued in children presenting with ventricular tachycardia.

Atypical presentation of plantar fasciitis secondary to soft-tissue mass infiltration.

This article describes a patient with plantar fascial pain who presented to the office of one of the authors. Physical examination and the patient's description of the history of symptoms revealed classic signs and symptoms of plantar fasciitis. The patient was treated with numerous conservative modalities, including ultrasound, nonsteroidal anti-inflammatory medications, trigger-point injections, over-the-counter orthoses, and stretching exercises. When the pain was not relieved by these conservative measures, magnetic resonance imaging of the area was performed. Visualization of the insertional area of the plantar fascia revealed a mass inferior to, as well as infiltrated into, the plantar fascia. Surgical excision of the lesion resulted in complete elimination of the patient's pain.

Diffuse intra-abdominal fibromatosis in association with bilateral ovarian fibromatosis and oedema.

A case of bilateral ovarian enlargement secondary to massive ovarian oedema with underlying intra-abdominal and pelvic fibromatosis is presented. Bilateral salpingo-oophorectomy and hysterectomy led to rapid progression of the intra-abdominal and pelvic fibromatosis, which was previously unsuspected. The case highlights the importance of recognizing massive oedema of the ovary as a distinct entity as it is a benign condition affecting young females and can be confused with ovarian neoplasm. In this case, the major morbidity was from the underlying diffuse intra-abdominal fibromatosis. Conservative management with prolonged bowel rest, total parenteral nutrition, and intravenous steroid and Tamoxifen successfully led to complete resolution of bowel obstruction from diffuse fibromatosis.

[Benign coloduodenal fistula caused by inflammatory pseudotumor of the colon]. / Fístula coloduodenal benigna por pseudotumor inflamatorio del colon.

We present a case of benign coloduodenal fistula, due to an inflammatory pseudotumor of the hepatic flexure of the colon, that was found included in a calcified hepatic cavity, presumably hydatic, that opened through a large fistulous orifice into the second duodenal portion. A barium enema satisfactorily demonstrated the colonic lesion and a fistulous tract. Although the preoperative diagnosis was of colonic neoplasm fistulized into the duodenum, the histopathological study of the surgical specimen did not demonstrate malignancy.

Sternomastoid tumor of infancy.

Sternomastoid tumor of infancy (SMTI) is the most common cause of neck mass in the perinatal period. We present seven children with this disorder, six studied prospectively. Ages at presentation ranged from 1 week to 4 weeks. Five had a history of birth trauma. Torticollis with facial asymmetry was seen in two. In six the diagnosis of SMTI was made clinically, and these patients were managed conservatively with massage and controlled stretching of the neck. Resolution of the neck mass, the torticollis, and the facial asymmetry occurred in all patients. Pathologic and radiographic findings are presented. We conclude that careful clinical assessment precludes the necessity of biopsy and emphasize the importance of conservative management of this transient problem.

Tumor-induced osteomalacia: long-term follow-up of two patients cured by removal of their tumors.

Two patients with mesenchymal tumors had osteomalacia associated with marked hypophosphatemia and renal phosphate wasting. Their serum calcium and parathyroid hormone levels were normal. The first patient also had aminoaciduria and glucosuria in addition to phosphaturia. Both patients were treated with very large doses of vitamin D2 and phosphate without improvement in the osteomalacia or normalization of the serum phosphate. Complete removal of a low-grade fibrosarcoma in the second patient and removal of an ossifying fibroma in the first patient resulted in a cure in both patients despite no further therapy with vitamin D or phosphate. The excessive aminoaciduria and glucosuria of the first patient also remitted. During a 14-year follow-up period, there has been no evidence of tumor recurrence, hypophosphatemia, or osteomalacia in either patient. The levels of 1,25-dihydroxycholecalciferol remain within the normal range in both patients.

Case of tumour rickets.

A 10-year-old boy, with widespread soft tissue tumours of bone, developed hypophosphataemic rickets due to impaired renal tubular reabsorption of phosphate. Biopsy of the largest tumour showed a nonosteogenic fibroma. We believe this boy is another example of 'tumour rickets', as other causes of rickets were excluded clinically and biochemically. Cases of rickets or osteomalacia associated with a tumour, have generally been reported to be cured by surgical removal of the tumour, implicating it as the cause of rickets or osteomalacia. Owing to the large number of tumours in this boy, surgical removal was not possible, and he required large doses of vitamin D, together with oral phosphate, before his rickets healed. It is suggested that the tumour produces a phosphaturic hormone.