Peritoneal lymphomatosis. A case report

ABSTRACT Introduction: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. Description of the case: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. Discussion: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. Conclusion: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.

Peritoneal lymphomatosis. A case report.

INTRODUCTION: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. DESCRIPTION OF THE CASE: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. DISCUSSION: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. CONCLUSION: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.

Schwannoma del plexo cervical en una mujer de 76 años / Schwannoma of the cervical plexus in a 76-year-old woman

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¿Cuáles son las claves de la granulomatosis linfomatoide con afectación extrapulmonar? / Which are the keys of the lymphomatoid granulomatosis with extrapulmonary disease?

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Quistes broncogénicos gástricos. Una localización inhabitual / Gastric bronchogenic cysts. An unusual location

Los quistes broncogénicos son lesiones benignas inhabituales que tienen su origen durante la embriogénesis del intestino proximal primitivo y se localizan principalmente en el pulmón. Están revestidos por epitelio cuboideo o columnar seudoestratificado y ciliado, conteniendo en su pared fibras elásticas, músculo liso, glándulas seromucinosas análogas a las bronquiales y/o cartílago. Los quistes broncogénicos gástricos son extremadamente raros, existiendo muy pocos casos descritos en la literatura. Suelen dar sintomatología compresiva, siendo el principal diagnóstico diferencial el GIST y el tratamiento habitual es resección quirúrgica del mismo

Bronchogenic cysts are rare benign lesions that arise during embryogenesis of the primitive proximal intestine and are located primarily in the lung. They are lined with cuboid or columnar pseudostratified and ciliated epithelium and have elastic fibers, smooth muscle, bronchial glands and cartilage in their walls. Gastric bronchogenic cysts are extremely rare with very few reported cases. Symptoms are usually caused by compression. GIST is the main differential diagnosis and the treatment is surgical resection

[Gastric bronchogenic cysts. An unusual location]. / Quistes broncogénicos gástricos. Una localización inhabitual.

Bronchogenic cysts are rare benign lesions that arise during embryogenesis of the primitive proximal intestine and are located primarily in the lung. They are lined with cuboid or columnar pseudostratified and ciliated epithelium and have elastic fibers, smooth muscle, bronchial glands and cartilage in their walls. Gastric bronchogenic cysts are extremely rare with very few reported cases. Symptoms are usually caused by compression. GIST is the main differential diagnosis and the treatment is surgical resection.

Hiperplasia muscular paratesticular (epididimaria) pseudotumoral. Descripción de 2 casos / Pseudotumoral paratesticular (epididymal) muscle hyperplasia. Two case reports

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Variante extracutánea de xantogranuloma juvenil en un adulto asociado a linfoma folicular / Extracutaneous variant of juvenile xanthogranuloma associated to follicular lymphoma in an adult patient

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Fibromixolipoma dendrítico infraclavicular: en el límite entre el lipoma de células fusiformes y el tumor fibroso solitario / Dendritic infraclavicular fibromyxolipoma: at the boundary between spindle cell lipoma and solitary fibrous tumour

El fibromixolipoma dendrítico (FMLD) es un tumor de partes blandas recientemente descrito, muy poco frecuente, de los cuales tan solo hay 27 casos reseñados en la literatura, ninguno con recidivas y/o metástasis tras su extirpación. Histológicamente está constituido por una proliferación de células pequeñas, estrelladas o fusiformes, en un estroma mixoide con abundantes haces de colágeno y áreas dispersas de tejido adiposo maduro. Dichas células proliferantes se tiñen, de forma característica, con CD34 y bcl-2, y muestran, entre otras aberraciones citogenéticas, deleción en la región 13q14.3. Presentamos un caso de FMLD localizado en la región infraclavicular, en un hombre de 69 años que refiere una masa de 5cm de diámetro, circunscrita y no dolorosa, de varios años de evolución, que tras su extirpación y durante un periodo de seguimiento de 2 años no ha mostrado recidivas ni metástasis. Describimos las características clinicopatológicas de esta entidad y sus diagnósticos diferenciales, haciendo una revisión amplia de la literatura (AU)

Dendritic fibromyxolipoma (DFML) is a rare soft tissue tumor recently described with only 27 cases reported in the literature. None of them have shown recurrence or metastasis after excision. Histologically it is composed of small stellate or spindle cells in a myxoid stroma with abundant collagen bundles mixed with mature adipose tissue. The proliferating cells typically show immunoexpression positive for CD34 and bcl-2. A cytogenetic analysis reveals deletion involving 13q14.3 region. We describe the first reported case to date located in the infraclavicular region. A 69 year old male with a painless mass well circumscribed and 5cm in size of several years of evolution, that after excision (2 years ago) he has not shown signs of recurrence or metastasis. This study reflects the clinicopathological features, differential diagnosis and a review of the literature of the DFML (AU)

[Dendritic infraclavicular fibromyxolipoma: At the boundary between spindle cell lipoma and solitary fibrous tumour]. / Fibromixolipoma dendrítico infraclavicular: en el límite entre el lipoma de células fusiformes y el tumor fibroso solitario.

Dendritic fibromyxolipoma (DFML) is a rare soft tissue tumor recently described with only 27 cases reported in the literature. None of them have shown recurrence or metastasis after excision. Histologically it is composed of small stellate or spindle cells in a myxoid stroma with abundant collagen bundles mixed with mature adipose tissue. The proliferating cells typically show immunoexpression positive for CD34 and bcl-2. A cytogenetic analysis reveals deletion involving 13q14.3 region. We describe the first reported case to date located in the infraclavicular region. A 69 year old male with a painless mass well circumscribed and 5cm in size of several years of evolution, that after excision (2 years ago) he has not shown signs of recurrence or metastasis. This study reflects the clinicopathological features, differential diagnosis and a review of the literature of the DFML.

Histological Features, p53, c-Kit, and Poliomavirus Status and Impact on Survival in Merkel Cell Carcinoma Patients.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare and aggressive malignancy from neuroendocrine cells in the skin. Despite being one of the most life-threatening of skin cancers, little is known about the potential signaling mechanism that drives carcinogenesis in MCC. The purpose of this study is to assess the impact of Merkel cell polyomavirus (MCPyV), p53, and c-kit on the histological features and clinical prognosis of MCC treated in our regional hospitals. METHOD: The design was a retrospective study. The specimens were taken between 1993 and 2013 in 2 referral hospitals of Southern Spain. Data were collected retrospectively and analyzed using SPSS software. RESULTS: Thirteen lesions from 13 subjects were included in the study. Positivity for c-kit was associated with the absence of MCPyV viral DNA (P = 0.048) and positivity for p53 (P = 0.002). More rate of mitoses per high-power field was presented significantly in those specimens with: positivity for c-kit (P = 0.046), positivity for p53 (P = 0.05), lesions with infiltrative growth pattern (P = 0.008), and lymphovascular invasion (P = 0.034). We observed an inverse relationship between p53 expression and MCPyV infection (Pearson's coefficient: -0.524; P = 0.046) and between c-kit expression and MCPyV infection (Pearson's coefficient: -0.548; P = 0.05), whereas the relationship was positive between p53 expression and c-kit expression (Pearson's coefficient: 0.884; P < 0.001). CONCLUSION: We conclude that presence of MCPyV DNA has no effect on overall survival. MCCs with p53 and c-kit expressions are associated with the absence of or low MCPyV DNA showing an inverse relationship. A multifactorial molecular pathogenesis where positivity for p53 and c-kit are associated with other mechanisms different than MCPyV (such as pro-mitotic factors) may lead to aggressive clinical behavior.

Molluscum Contagiosum Infection Involving a Benign Epidermoid Cyst in an Immunocompetent Patient.

A 78-year-old man presented with a round- to oval-shaped nodule on his right eyebrow. The lesion first developed 5 years ago as a small solitary white nodule and subsequently enlarged over the past 2 years. His medical history was unremarkable. Clinical examination revealed a 2-cm round to oval dome-shaped yellowish nodule with a dimple on the top center (Figure 1). No similar lesions were found elsewhere. With a clinical suspicion of sebaceous carcinoma, an excision of the lesion was performed under local anesthesia. The histopathologic analysis showed an epidermal cyst containing molluscum bodies along the keratin inside the cyst (Figure 2). With these findings, the diagnosis of molluscum contagiosum (MC) infection into an epidermoid cyst was made. Neither recurrence nor new similar lesions were observed at follow-up.

[Solitary fibrous hemangiopericytoma of atypical location: importance of immunohistochemical study]. / Tumor fibroso solitario hemangiopericitoma de localización atípica: importancia del estudio inmunohistoquímico.

BACKGROUND: The rare cutaneous solitary fibrous tumor was initially described in the thoracic cavity in relation to the pleura and subsequently been associated with other serous membranes. It has been described in other extraserosal locations including the skin. Knowledge of its existence along with fairly typical histological features and the immunohistochemical expression pattern with intense positivity for CD34 allow the increasing diagnosis of this condition, which suggests that these cases were not previously diagnosed as such. CLINICAL CASE: We report the case of a 43 year-old male with a painless nodule in the first left finger pad clinically suggestive of pyogenic granuloma or nodular melanoma, which was diagnosed by excisional biopsy and immunohistochemical study as a solitary fibrous tumor. DISCUSSION: Only 11 cases of cutaneous solitary fibrous tumor have been published in the following locations: head, cheek, thigh, chest, back and nose. Our work describes the first case of cutaneous solitary fibrous tumor in the hand. The solitary fibrous tumor derived from mesenchymal cells expresses CD34 and hence its presentation in any location. In our case it was in the hand. It explains the problems encountered in the clinical differential diagnosis with other tumors as nodular melanoma, pyogenic granuloma, giant cell tumor of tendon sheath, fibroma, benign peripheral nerve sheath tumors, etc. As we consider the histology, differential diagnosis should be made with other tumors that also express CD34. CONCLUSIONS: Solitary fibrous tumors derived from mesenchymal cells express CD34 and hence its presentation in any location. In our case it was in the finger pad.

Antecedentes: el tumor fibroso solitario es un tumor poco común. Anteriormente se suponía que afectaba sólo la cavidad torácica, en especial la pleura; posteriormente, se relacionó con otras membranas serosas y se observó en diversas localizaciones extraserosas, entre ellas la piel. El conocimiento de este tumor, junto con el aspecto histológico característico y el patrón de expresión inmunohistoquímica con intensa positividad para CD34 permiten que cada vez se diagnostique con mayor frecuencia. Caso clínico: se comunica el caso de un varón de 43 años de edad con un nódulo indoloro en el pulpejo del primer dedo izquierdo, que sugería clínicamente un melanoma nodular o granuloma piógeno. Mediante biopsia excisional y estudio inmunohistoquímico se diagnosticó como tumor fibroso solitario. Discusión: hasta la fecha se han publicado 11 casos de tumores fibrosos solitarios cutáneos, localizados en cabeza, mejilla, muslo, pecho, espalda y vestíbulo nasal. El caso que se comunica constituye la primera lesión de estas características que afecta la mano. El diagnóstico clínico diferencial del tumor fibroso solitario incluye otros tumores como: melanoma nodular, granuloma piógeno, tumor de células gigantes tenosinovial, fibroma y tumor de vaina de nervio periférico benigno. En cuanto a la histología, se planteó el diagnóstico diferencial con otras neoplasias que también expresan CD34. Conclusiones: el tumor fibroso solitario deriva de células mesenquimatosas y expresa CD34, lo que explica su aparición en cualquier localización, como en este caso, que fue en el pulpejo del quinto dedo.