Primary pulmonary Hodgkin lymphoma
(PPHL) is a rare disease. Herein, we report a case of PPHL with diagnostic concerns encountered during initial evaluation which is of paramount importance to keep the differential diagnosis in cases with high index of sus- picion for this rare entity. (Source: Rare Tumors)
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Autopsy analysis may contribute to establish actual incidence of second primary malignancies in myeloma
This study aimed to examine SPMs using autopsy reports. Ninety-one cases of MM autopsied at our institution from 1979 to 2013 were analyzed. Median age of autopsied patients was 64.1 years, and proportion of male/female was 59/32. Autopsy was performed in 35.3% of patients died of MM. There were five cases of SPMs with a median confirmation time of 38 (12-132) months from the diagnosis of MM. In three of the five patients, the diagnosis of SPMs was established at autopsy. One case was of myelodysplastic syndrome, and the others were of non-hematological malignancies. The annual risk of SPM estimated using the Kaplan-Meier ...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
T-cell/histiocyte-rich large B-cell lymphoma presenting as a primary central nervous system lymphoma
Primary central nervous system (PCNSL) lymphoma is an aggressive extranodal non-Hodgkin lymphoma, and most cases are classified as diffuse large B-cell lymphoma (DLBCL) by histology. T-cell/histiocyte-rich large B-cell lymphoma (TCRLBCL) represents a distinct subtype of diffuse large B-cell lymphoma and is characterized by the presence of scattered large neoplastic B-cells in a background of abundant T-cells and histiocytes. This is in contrast to the dense perivascular cuffing of neoplastic B-cells in classic DLBCL. T-cell/histiocyte-rich large B-cell lymphoma should be considered in PCNSL cases in which neoplastic B-cell...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Excellent outcome of immunomodulation or Bruton’s tyrosine kinase inhibition in highly refractory primary cutaneous diffuse large B-cell lymphoma, leg type
We report a case of PCDLBCL-LT of activated B-cell subtype characterized by multiple local relapses in the legs, initially, and systemic relapses about seven years after the diagnosis. Local relapses were sensitive to radiation therapy. Cutaneous and systemic relapses responded well to immunomodulatory therapy with lenalidomide followed by Bruton’s tyrosine kinase inhibition with ibrutinib. Ibrutinib is the only treatment that resulted in long-lasting complete remission. Lenalidomide and especially ibrutinib appear to have a significant activity against this lymphoma and should be incorporated in the treatment of this re...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
The effectiveness of bevacizumab in radionecrosis after radiosurgery of a single brain metastasis
We present a case of a 73-years-old women affected by a primary non-small cell lung cancer with a single brain metastasis treated with radiosurgery. Two years after radiosurgery the patient referred neurological symptoms and a brain magnetic resonance confirmed the presence of RN. The patient refused surgical decompression so underwent at the treatment with bevacizumab 7.5 mg/kg/2 weeks for a total of 4 cycles. After two months of treatment the patient reported strumental and clinical improvement. Ten months after bevacizumab discontinuation the patient experienced a recurrence of RN with evident clinical manifestation and...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Giant scrotal fibrolipoma
We report a case of a 55-year-old male complaining for a slow-growing, painless mass in his left hemiscrotum. Imaging with ultrasonography and magnetic resonance imaging was inconclusive regarding the nature of the tumor and the tumor was excised, sparing the testis. The surgical specimen was a well-defined, yellowish white, solid, and firm mass, measuring 19.5×7×5 cm. There was no cytological atypia or mitosis and no lipoblasts recognized. On immunohistochemistry, MDM2 and CDK4 were not expressed. The histopathology report was fibrolipoma of the scrotum. To the best of our knowledge, this is only the fourth case of fibr...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Small cell carcinoma of the urinary bladder: KIT and PDGFRA gene mutations
Primary small cell carcinoma of the urinary bladder is very rare. A 72-year-old was admitted to our hospital because of hematuria and dysuria. Cystoscopy revealed a bladder full of multiple, solid and papillary tumors. Biopsies from the deep and papillary tumors were taken. Histologically, tumor was pure small cell carcinoma. Immunohistochemically, the tumor cells were positive for cytokeratin, chromogranin, synaptophysin, neuron-specific enolase, CD56, CD117 and Ki67 (labeling 70%). The tumor cells were negative for CK7, CK20, CD3, CD20, LCA, CDX2, uroplakin, thyroid transcription factor 1, PSA and p63. Metastatic workup ...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Dermatofibrosarcoma protuberans with lung metastasis requiring pneumonectomy
We report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted therapy with Imatinib. The patient is alive with stable disease after thirty months of the metastases diagnoses. Dermatofibrosarcoma protuberans metastasizes very rarely, and when it does, it is usually either after local recurrence or whenever fibrosarcomatous transformation is found in the histopathological analysis, which con...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Subcutaneous axillary and scalp metastases from non-gynecological retroperitoneal leiomyosarcoma: an unusual presentation after surgical resection
We present a case of 72-year-old male with scalp nodule and subcutaneous swelling in left posterior axillary fold. Fine needle aspiration cytology from both these sites revealed a sarcoma, which was positive for Smooth Muscle Actin and negative for S100 on cell block immunohistochemistry (IHC). The past history revealed surgical resection of a retroperitoneal mass in 2010 which was diagnosed on histopathology and IHC as leiomyosarcoma. A final diagnosis of metastatic deposits from leiomyosarcoma was made. Retroperitoneal leiomyosarcoma presenting as scalp and subcutaneous metastasis is an unusual presentation. Adequate cli...
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Spontaneous regression of primitive Merkel cell carcinoma
We describe the case of a 89-year-old male patient who presented two MCC lesions of the scalp. Following biopsy the lesions underwent complete regression with no clinical evidence of residual tumor up to 24 months. The current knowledge of MCC and the other cases of spontaneous regression described in the literature are reviewed. (Source: Rare Tumors)
Source: Rare Tumors - December 29, 2015 Category: Cancer & Oncology Source Type: research
Management of metastatic apocrine hidradenocarcinoma with chemotherapy and radiation
Hidradenocarcinoma is a rare aggressive form of cutaneous adnexal skin carcinoma originating from the sweat gland. Due to its low incidence, prognostic and treatment strategies are still being explored both for primary and advanced disease. This tumor most often presents as either solid or cystic appearing subcutaneous nodules, which may be associated with pruritus or ulceration. To date the mainstay of treatment for local disease has been surgical excision; however, the paucity of historical data available has shown that these tumors often behave aggressively with high rates of local recurrence, metastasis, and poor overa...
Source: Rare Tumors - October 5, 2015 Category: Cancer & Oncology Source Type: research
Metastatic renal cell carcinoma masquerading as a primary ovarian mass in a post-operative case of meningioma and renal cell carcinoma
We present a case of metastatic renal cell carcinoma diagnosed in a 48-year-old female, who had renal cell carcinoma of the right kidney and right sphenoid wing meningioma of transitional type. (Source: Rare Tumors)
Source: Rare Tumors - September 30, 2015 Category: Cancer & Oncology Source Type: research
A case of endometrioid adenocarcinoma originating from the serous surface of the small intestine
Malignant transformation of endometriosis has been extensively described in the literature. However, extragonadal endometrioid adenocarcinoma, either de novo or arising from malignant transformation of endometriosis, is rare. The present case report describes a patient with endometrioid adenocarcinoma on the serous surface of the small intestine. A 25- year-old female with no history of endometriosis was referred to our hospital with an intrapelvic tumor. An internal examination, ultrasound, and magnetic resonance imaging revealed a round mass approximately 80 mm in diameter; however, identification of the affected organ w...
Source: Rare Tumors - September 30, 2015 Category: Cancer & Oncology Source Type: research
Carcinosarcoma with choriocarcinomatous and osteosarcomatous differentiation in a patient with juvenile polyposis syndrome
We describe the case of a 12-year-old boy with JPS associated with an abdominal tumor. Histological sections of the abdominal tumor showed components of adenocarcinoma, osteosarcoma, and choriocarcinoma. Immunohistochemistry was AE1/AE3, CK7, HCG and SALL4 positive. Juvenile polyposis syndrome patients are at increased risk of colorectal adenocarcinoma. However, we present a case of an adenocarcinoma associated with other unusual components. This association has not been reported before. (Source: Rare Tumors)
Source: Rare Tumors - September 30, 2015 Category: Cancer & Oncology Source Type: research
Synchronous pulmonary malignancies: atypical presentation of mantle cell lymphoma masking a lung malignancy
We present a case of a pleural space malignancy masked by an atypical presentation of mantle cell lymphoma. Our patient presented with a large pleural effusion and right sided pleural studding, initially attributed to a new diagnosis of mantle cell lymphoma. Rare atypical epithelial cells were also seen amongst the clonal population of lymphocytes. The patient lacked systemic manifestations of mantle cell lymphoma and did not improve with chemotherapy. A pleural biopsy ultimately revealed the presence of an undifferentiated carcinoma, favoring a lung primary. A discussion of synchronous pleural space malignancies involving...
Source: Rare Tumors - September 30, 2015 Category: Cancer & Oncology Source Type: research
