Carcinoma epidermoide metastásico asociado a epidermólisis ampollosa distrófica / Metastasic squamous cell carcinoma in dystrophic epidermolysis bullosa

Caso clínico: femenino de 18 años con diagnóstico de epidermolisis ampollosa distrófica (EAD) quién desarrolló una neoformación nodular sobre una úlcera crónica. Se diagnosticó carcinoma epidermoide (CE) invasor al que se realizó resección. Sin embargo, 5 meses después del tratamiento quirúrgico presentó metástasis a ganglios, pulmón e hígado con desenlace fatal. Comentarios: el CE es la causa más importante de muerte en pacientes con EAD. Suele ser agresivo y metastásico. Se recomienda una vigilancia cada 3 a 6 meses para realizar diagnóstico y tratamiento oportunos (AU)

Case report: 18-year-old female patient with dystrophic epidermolysis bullosa (DEB) who developed a tumor over a chronic ulcer. She was diagnosed with invasive squamous cell carcinoma (SCC) and underwent surgical resection. However, 5 months later she presented metastases to the lymph nodes, lung and liver with a fatal outcome. Comments: SCC is the most important cause of death in patients with DEB. It is usually aggressive and metastatic. Surveillance every 3 to 6 months is recommended for prompt diagnosis and treatment (AU)

Cutaneous metastasis of unknown primary presenting as massive and invasive abdominal lesion: an elective approach

Abstract: We describe herein what is to our knowledge the first reported case of an invasive cutaneous metastasis with unknown primary, electively treated solely with electrochemotherapy. We describe a female patient with a large, invasive and painful lesion in her hypogastric region, extending up to the pubic area. The cutaneous biopsy and instrumental and laboratory analyses, all failed to reveal the primary site. A final diagnosis of cutaneous metastasis with unknown primary was made and treatment was performed with electrochemotherapy. Our case highlights the importance of interdisciplinary choices in clinical practice to cope with the lack of a primary site and to improve quality of life, since no standardized therapy exists for these classes of patients.

Cutaneous metastasis of unknown primary presenting as massive and invasive abdominal lesion: an elective approach with electrochemotherapy.

We describe herein what is to our knowledge the first reported case of an invasive cutaneous metastasis with unknown primary, electively treated solely with electrochemotherapy. We describe a female patient with a large, invasive and painful lesion in her hypogastric region, extending up to the pubic area. The cutaneous biopsy and instrumental and laboratory analyses, all failed to reveal the primary site. A final diagnosis of cutaneous metastasis with unknown primary was made and treatment was performed with electrochemotherapy. Our case highlights the importance of interdisciplinary choices in clinical practice to cope with the lack of a primary site and to improve quality of life, since no standardized therapy exists for these classes of patients.

Subcostal transversus abdominis plane phenol injection for abdominal wall cancer pain.

A subcostal transversus abdominis plane (TAP) phenol injection was performed on a patient with refractory cancer pain due a metastatic involvement of the abdominal wall. A diagnostic block with local anesthetic was performed under ultrasound guidance (USG), resulting in a decrease of 80% and 100% in dynamic and static visual analog scale (VAS) for pain, respectively, for 20 hours. A phenol injection was then performed under USG. The patient reported 70% and 100% reduction in the dynamic and static VAS for pain and had a 50% decrease in the opioid requirement that was maintained for 2 months. TAP blocks offer an interesting tool for either diagnosis or therapeutic purpose in chronic pain management. USG provides an optimal approach to soft-tissue lesions where fluoroscopy techniques are not useful.

Video-assisted left inguinal lymphadenectomy for penile cancer.

BACKGROUND: Penile cancer is a rare disease, most commonly encountered in developing countries. It constitutes 0.4% of cancers in U.S. men and 2.1% in Brazil, with the highest prevalence in the North and Northeast regions. Inguinal lymph node metastasis of penile cancer occurs in 20 to 40% of patients and is an important predictor of cancer-specific mortality. The preferred diagnostic and therapeutic tool to assess the regional lymph nodes is a lymphadenectomy which can, in addition to establishing staging, offers curative potential. MATERIALS AND METHODS: A 44 years old man, previously to underwent a partial penectomy for penile cancer, whose pathology showed a moderately differentiated squamous cell carcinoma with neural and angiolymphatic invasion and negative surgical margins. The pathologic stage of the primary tumor was pT3NxMx. Following a one month course of oral antibiotics, the patient underwent a video-assisted bilateral inguinal lymphadenectomy. In the present video, we highlight the left video-assisted inguinal lymphadenectomy. RESULTS: Seventeen lymph nodes were dissected on the left side, two of them positive for cancer without extracapsular extension. On the right side, fourteen lymph nodes were dissected and one was positive for cancer with extracapsular extension, and the patient underwent based on these pathological findings a pelvic lymphadenectomy, which was similarly conducted using a video-assisted laparoscopic approach. CONCLUSIONS: The conventional open lymphadenectomy has a morbidity that can approach 50% in the current series, despite on the refinements in technique. The video-assisted endoscopy is a recent technique aiming to decrease this inherent complication rate promoting a lymph node resection rate which may be equivalent to the open procedure. This video confirms its feasibility, reduced morbidity, and cancer control efficacy.

Late diagnosis of nodular melanoma of the foot in a 74-year-old Brazilian man / Diagnóstico tardío de melanoma nodular del pie en un varón con 74 años

The incidence and mortality of malignant melanoma is increasing. Its early diagnosis can contribute to a favorable prognosis. We report a 74-year-old black man with a dark brown mole in the right plantar area, that grew slowly in the last decade. A biopsy of the lesion revealed a nodular malignant melanoma. A wide excision of the tumor was performed 18 months ago, followed by chemotherapy for nine months. At admission, the patient was debilitated, and presented with numerous nodules in the lower limb and suprapubic area. Imaging studies disclosed chest and abdominal nodules. The patient died 13 days after admission.

Melanoma maligno constituye una importante preocupación debido al incremento de la incidencia y mortalidad. El diagnóstico precoz de esta malignidad puede contribuir para prognósticos favorables. Se describe el diagnóstico tardío de melanoma nodular del pie en un varón afro-brasileño con 74 años de edad. El propósito es aumentar el índice de sospecha acerca de esta infrecuente localización del tumor de la piel, y estimular a los médicos de cuidado primario para realizar examen minucioso del cuerpo entero, contribuyendo a la detección temprana de los cánceres de piel.

Extraneural metastases in medulloblastoma.

Medulloblastoma is the most common childhood malignant tumor of central nervous system, but it may also occur in adults. It presents high invasive growth with spreading of tumor cells into the leptomeningeal space along the neuroaxis early in the course of the disease. Extraneural metastases are rare but frequently lethal, occurring only in 1 to 5% of patients, and are related, in the most of cases, to the presence of ventriculoperitoneal shunt. Here we characterize the clinical profile of five cases of medulloblastoma with systemic spreading of tumor cells, also comparing them to cases already described in the literature.

Late diagnosis of nodular melanoma of the foot in a 74-year-old Brazilian man.

The incidence and mortality of malignant melanoma is increasing. Its early diagnosis can contribute to a favorable prognosis. We report a 74-year-old black man with a dark brown mole in the right plantar area, that grew slowly in the last decade. A biopsy of the lesion revealed a nodular malignant melanoma. A wide excision of the tumor was performed 18 months ago, followed by chemotherapy for nine months. At admission, the patient was debilitated, and presented with numerous nodules in the lower limb and suprapubic area. Imaging studies disclosed chest and abdominal nodules. The patient died 13 days after admission.

Extraneural metastases in medulloblastoma / Mestástases extraneurais em moduloblastoma

Medulloblastoma is the most common childhood malignant tumor of central nervous system, but it may also occur in adults. It presents high invasive growth with spreading of tumor cells into the leptomeningeal space along the neuroaxis early in the course of the disease. Extraneural metastases are rare but frequently lethal, occurring only in 1 to 5 percent of patients, and are related, in the most of cases, to the presence of ventriculoperitoneal shunt. Here we characterize the clinical profile of five cases of medulloblastoma with systemic spreading of tumor cells, also comparing them to cases already described in the literature.

O meduloblastoma é o tumor maligno mais frequente do sistema nervoso central na infância, mas também pode ocorrer em adultos. Ele apresenta crescimento altamente invasivo com disseminação de células tumorais ao longo do neuroeixo precocemente no curso da doença. Metástases extraneurais são raras mas frequentemente letais, ocorrendo apenas em 1 a 5 por cento dos pacientes, e estão relacionadas, na maioria dos casos, a presença de derivação ventriculperitoneal. Neste artigo ,apresentamos o perfil de cinco casos de meduloblastoma com disseminção sistêmica das células tumorais, comparando-os com os casos já descritos na literatura.

Incidental gallbladder cancer: analysis of surgical findings and survival.

BACKGROUND AND OBJECTIVES: Incidental gallbladder cancer (IGBCA) has risen worldwide and its prognosis depends on complete radical cholecystectomy (CRC). This study evaluated surgical findings during re-operation and survival of patients with IGBCA. METHODS: Demographics, surgical treatment, staging, and survival data for all IGBCA patients who underwent surgery at Instituto Oncológico Fundación Arturo López Pérez (FALP) between 2000 and 2008 were analyzed. Differences between groups were analyzed by Student's t-test, Mann-Whitney, chi-square, or Fisher log-rank tests. RESULTS: Forty-nine patients were studied (38 women/11 men, median age = 58 years). Pathology reports from cholecystectomy showed that 32 patients had a T2 tumor and 12 had positive resection margin. Thirty-six patients underwent surgical re-exploration and 20 underwent CRC; 10 with (+) residual disease and 10 with (-). For patients with at least T1b tumor, median survival was 28 months and 5-year disease-specific survival (DSS) was 29%. The 3-year DSS was 64% for CRC (-), 30% for CRC (+), and 8% for non-resected cases (P < 0.007). The 3-year DSS was better for patients with stage Ib than those with stages II and IV (P < 0.007). CONCLUSIONS: Patients with IGBCA have a high chance of intra-abdominal metastases or local residual disease. In CRC patients, intra-abdominal metastases were associated with a worse prognosis.

Nódulo de la hermana María José" en tumor de ovario gigante / \"Sister María José\" nodule in giant ovary tumor

Se presenta el caso de una paciente con un gran tumor abdominal y nódulo umbilical. La citología aspirativa con aguja fina confirmó la presencia de lesión metastásica por adenocarcinoma con ßreas papilares de alto grado, lo que unido a la clínica y al resto de las investigaciones permitió establecer que se trataba de una neoplasia de ovario con metástasis en hígado, bazo y ganglios. Concluimos que ante el hallazgo clínico de un ganglio umbilical es necesario tomar muestra para estudio histológico, lo que facilita el diagnóstico y la conducta terapéutica

This is the case of a patient presenting with a large abdominal tumor and umbilical nodule. The fine-needle aspiration cytology confirmed the presence of a metastatic lesion due to adenocarcinoma with a high degree papillary areas and it joined to clinics and remainder researches allowed to establish the presence of ovarian neoplasm in liver, spleen and ganglia. We concluded that in the face of a clinical finding of an umbilical ganglion, it is necessary to take a sample for histological study allowing the diagnosis and the therapeutical behavior

Fine needle aspiration biopsy of gastrointestinal stromal tumor presenting as an umbilical mass (Sister Mary Joseph's Nodule).

The Sister Mary Joseph (SMJ) nodule is a clinical sign of metastatic cancer involving the umbilicus. The vast majority of these instances represent adenocarcinomas arising from ovarian or colorectal primaries. We present a patient who presented with ascites and the SMJ lesion that turned out to be a metastatic gastrointestinal stromal tumor after fine needle aspiration biopsy was performed. The lesion was subsequently histologically confirmed. Gastrointestinal stroma tumor involving the umbilicus is exceedingly uncommon and only rarely presents in this fashion. The cytomorphological features, differential diagnosis, and comparison with the tissue specimen are made.

[Sarcomas of the vulva. Report of 2 cases]. / Sarcomas de la vulva. Informe de dos casos.

We report two cases of vulvar sarcomas that are our Institutional experience in 29 years. The first case was a teenager of 14 years-old with a low grade leiomyosarcoma surgically treated. Along a 22 years follow-up the disease has had four local recurrences of more than 5 cm each one: two after surgery and two after surgery plus chemotherapy and surgery plus radiotherapy respectively. She is alive disease evidence after two years from the last combined treatment. The second one, was a 26 years-old patient with a malignant schwannoma of 12 cm in diameter treated with combined radical surgery, radiotherapy, and chemotherapy. She is alive and without disease evidence 52 months after surgery. We emphasized that these tumors are very rare and the fact that the first patient is the youngest and with more years of follow up according the bibliography consulted. Treatment of vulvar sarcomas is radical local excision followed mainly by radiotherapy with infiltrating margins. The value of postoperative adjuvant chemotherapy is uncertain. According to the natural history and behavior of vulvar sarcomas, we conclude that the elective treatment of these tumors should be carry out in institutions of high level.

Increased plasma atrial natriuretic factor in catecholamine-producing tumor patients.

The aim of this study was to evaluate plasma levels of ANF in patients with catecholamine-secreting tumors with and without hypertension and to relate ANF secretion to levels of plasma and urinary catecholamines and blood pressure. Twenty-one pheochromocytoma (15 with sustained, 6 with paroxysmal hypertension), 6 neuroblastoma (1 hypertensive) patients and 28 aged-matched controls were studied in basal conditions. Plasma and urinary norepinephrine (NE),epinephrine (E), dopamine (DA) and DOPA were determined by HPLC-ED and plasma ANF by RIA. Both neuroblastoma and pheochromocytoma patients had significantly higher plasma ANF levels than controls. Neuroblastomas showed higher ANF concentration than pheochromocytomas. No differences were found in plasma ANF between hypertensive and normotensive patients. Pheochromocytomas with ANF levels within the normal range had plasma and urinary NE and urinary DA and DOPA levels significantly higher than patients with high ANF. Plasma ANF levels were unrelated to systolic or diastolic blood pressure or heart rate. A negative correlation between plasma ANF and urinary DA was found only in the patients groups. In conclusion, plasma ANF was increased in pheochromocytoma and neuroblastoma patients. Our data suggest that the excessive catecholamine secretion is not responsible for the increased ANF secretion in these patients. The significance of the relationships among plasma ANF and urinary and plasma catecholamines requires further investigation.

Abdominal recurrence of osteogenic sarcoma: a case report.

PURPOSE: Nonpulmonary metastases from osteogenic sarcoma are rare. A patient had a localized osteogenic sarcoma of the left femur which recurred in the abdomen, a previously unreported metastatic site. PATIENT AND METHODS: An 18-year-old boy was treated for osteosarcoma. He had abdominal pain, vomiting, weight loss, and symptoms of intestinal obstruction at the time of relapse. RESULTS: The patient had diffuse widespread intraabdominal osteogenic sarcoma as the only site of initial recurrence. Abdominal computerized tomography revealed ascites and calcified masses on the hepatic and peritoneal surfaces. Laparoscopic visualization of the abdomen showed hemorrhagic ascites and multiple calcified tumor on the peritoneum, diaphragm, and liver. A biopsy of a representative lesion confirmed the diagnosis of osteogenic sarcoma. The patient died from progressive disease. CONCLUSION: As the initial treatment for patients with osteogenic sarcoma is intensified, the pattern of metastases may change. Unusual sites of recurrence such as in this patient may become more prevalent. A clinical presentation of an acute abdomen in a patient previously treated for osteogenic sarcoma should prompt suspicion of intraabdominal recurrence.