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1.  Soft Tissue Sarcomas of the Kidney 
Rare Tumors  2015;7(1):5635.
Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skeleton in young adults. The prognosis is poor with a 5-year disease-free survival rate of 45-55%. The primary therapeutic approach is surgical resection. Most randomized studies assessing adjuvant chemotherapy for all types of localized soft tissue sarcomas did not show statistically significantly better overall survival times after chemotherapy, although they did show longer progression-free survival. We report on two cases of primary renal synovial sarcoma and one case of PNET of the kidney.
PMCID: PMC4387353  PMID: 25918607
synovial sarcoma of the kidney; primitive neuroectodermal tumor of the kidney; rare mesenchymal tumor
2.  Posterior Mediastinal Capillary Hemangioma Misdiagnosed as Neurofibromas: A Rare Case Report and Review of the Literature 
Rare Tumors  2015;7(1):5639.
Capillary hemangioma is a common benign tumor which can occur everywhere in the whole body, however its occurrence in posterior mediastinum is extremely rare, and to the best of our knowledge less than 20 cases have been reported in the English literature so far. Here in we report a 65-year-old lady who presented with prolonged cough and diagnosed to have a posterior mediastinal mass. Before operation, according to the site of tumor, it has been diagnosed as neurofibroma. It is very important to consider hemangioma before operation to reduce surgical complications, and it should be in the differential diagnosis of posterior mediastinal masses.
PMCID: PMC4387354  PMID: 25918608
mediastinum; capillary hemangioma
3.  Primary Gastric Hemangioblastoma: Report of a Case 
Rare Tumors  2015;7(1):5679.
Capillary hemangioblastoma (CHB) is a benign, highly vascularized tumor that generally occurs in central nervous system either in the setting of von Hippel-Lindau (VHL) disease or, more often, as a solitary sporadic lesion that is increasingly recognized in extraneural sites. We present the case of a 18 year-old man with abdominal pain, nausea and hematemesis, the endoscopy showed polypoid tumor bleeding of 5 cm in gastric antrum. The patients had not signs of VHL disease and was subjected to subtotal gastrectomy and referred to our institution. To our knowledge this is the first reported case of CHB occurring in stomach.
PMCID: PMC4387357  PMID: 25918611
Von Hippel-Lindau; hemangioblastoma
4.  Primary Gastric Melanoma: Case Report of a Rare Malignancy 
Rare Tumors  2015;7(1):5683.
We report the case of a 64-year-old white male who presented to his primary care physician with complaints of fatigue. Physical exam was unremarkable and laboratory studies revealed profound anemia, for which the patient received a transfusion. Esophagogastroduodenoscopy revealed a bleeding mass in the proximal stomach that was histologically determined to be malignant melanoma, with immunohistochemical staining demonstrating positivity for SOX10, S100, MART-1, and HMG-45. After an extensive dermatological exam no other primary lesion was identified. Whole body positron emission tomography (18-FDG-PET/CT) demonstrated pathologic uptake only in the area of the proximal stomach. For this reason, primary gastric melanoma was suspected in this patient. The patient underwent subtotal gastrectomy with mass excision followed by Roux-en-Y reconstruction. Very few cases of primary gastric melanoma have been reported. We report this case and present diagnostic criteria for primary non-cutaneous melanoma and discuss potential non-surgical therapies.
PMCID: PMC4387358  PMID: 25918612
melanoma; surgery; gastric; gastrointestinal
5.  Giant Cells Glioblastoma: Case Report and Pathological Analysis from This Uncommon Subtype of Glioma 
Rare Tumors  2015;7(1):5634.
Glioblastoma multiforme (GBM) is the most common glial tumor of the brain system; nevertheless, the giant cell (GC) subtype is uncommon. Recent reviews report for an incidence of 1% in adults and 3% in children. The GCs usually have a better prognosis than GBM and also an increasing long-term survival rate. It is known that the diagnosis of this tumor is due to its histological findings and patterns, such as the unusual increased number of giant cells. Unfortunately, due to its rarity, the immunohistochemical and cytogenetical analysis of this tumor is not well known. Some authors also suggest that there are few subtypes of GCs and their patterns of aggressiveness could be due to cytogenetical markers. It is recognized that maximum safe resection treatment and adjuvant radiotherapy can improve survival rate (5-13 months) similar to GBM patients.
PMCID: PMC4387352  PMID: 25918606
glioblastoma multiforme; giant cell glioblastoma; genetic; immunohistochemical; long-term survival
6.  A Rare Occurrence of Neonatal Nephroblastoma in Sub-Saharan Africa: A Case Report and Management in a Resource-Constrained Region 
Rare Tumors  2015;7(1):5657.
Neonatal nephroblastoma has been rarely reported in African neonate. A premature newborn (a 5-day-old male) was transferred with a history of neonatal abdominal mass. Ultrasonography revealed 75×46 mm, well-defined mass with mixed echogenicity replacing the right kidney. The patient underwent right radical nephrectomy and the tumor was confirmed to be a blastemal predominant Wilms’ tumor by the histopathological examination and has an unfavorable prognosis. The child died secondary to multiple organ failure, three days after surgery. Our case report serves to remind us the need to bear in mind the possibility of the diagnosis of neonatal nephroblastoma in neonate with renal mass.
PMCID: PMC4387355  PMID: 25918609
neonates; renal tumors; nephroblastoma; Kinshasa; the Democratic Republic of Congo
7.  A Case of Lymphoepithelioma-like Carcinoma in the Uterine Cervix 
Rare Tumors  2015;7(1):5688.
Lymphoepithelioma-like carcinoma occurring in the reproductive organs is a rare variant of squamous cell carcinoma, and this tumor of the uterine cervix accounts for 0.7% of all primary cervical uterine neoplasms. Associations with Epstein-Barr virus (EBV) and human papilloma virus (HPV) have been demonstrated in some studies. Some investigators suggested that EBV has an important role in the initiation of lymphoepitheliomalike carcinoma in Asian women. Here we report the case of a 45-year-old Japanese woman, gravida 2 and parity 2. She was admitted due to severe atypical genital bleeding caused by uterine cervical cancer. A >60-mm tumor was detected at the uterine cervix, and no distal metastasis or swallowing of lymph nodes was revealed by magnetic resonance imaging and a computed tomography scan. The cervical cancer stage FIGO Ib2 was diagnosed, and a radical hysterectomy was performed for this malignant tumor. The in situ hybridization for EBV was negative. HVP infection was strongly suspected because the squamous cell carcinoma was observed macroscopically in the uterine cervix. The prognosis of uterine lymphoepithelioma-like carcinoma is thought to be better than those of other cervical cancer types, but careful follow-up at fixed intervals is recommended. The patient has been followed up for 4 months since her surgery, and no evidence of recurrence has been detected.
PMCID: PMC4387360  PMID: 25918614
uterine cervical cancer; lymphoepithelioma-like carcinoma; squamous cell carcinoma
8.  Comparative Outcome of Computed Tomography-guided Percutaneous Radiofrequency Ablation, Partial Nephrectomy or Radical Nephrectomy in the Treatment of Stage T1 Renal Cell Carcinoma 
Rare Tumors  2015;7(1):5583.
Renal cell carcinoma (RCC) accounts for 3% of all cancers in adults. The indications for Radiofrequency Ablation (RFA) for renal carcinomas include T1a (tumor 4 cm or less, limited to the kidney), elderly patients, renal impairment, comorbidities, poor surgical candidate, and multiple bilateral renal masses. We retrospectively reviewed medical records, specifically investigating the indications, complications and outcomes of RFA and nephrectomy for treatment of RCC in a tertiary medical center with a predominantly Hispanic patient population. Forty-nine patients with RCC were evaluated. Nine patients had RFA, 9 had partial nephrectomy and 31 had radical nephrectomy. All patients among the 3 groups had stage T1N0M0 RCC at diagnosis. Tumor recurrence was observed in 2 (22%) patients that had RFA, one (11%) patient that had partial nephrectomy and no patients that had radical nephrectomy. One patient had recurrence of the tumor at the opposite kidney pole from the initial RFA site 4 years later. This particular patient did not have any tumor recurrence at the site of the initial RFA. A second RFA was performed on the recurrent tumor with no recurrence upon subsequent follow up visits. The second patient had recurrence of the RCC on 1 year follow that was discovered to be sarcomatoid RCC, which is an aggressive type with a poor prognosis. Our results support the clinical utility of RFA in patients with stage T1 RCC who are poor surgical candidates or those with reduced renal function. The clinical utility of RFA as an equally effective approach when compared to partial nephrectomy in patients with stage T1 RCC that meet strict indications for the procedure. The treatment choice should be individualized and based on the characteristics of the renal tumor such as size, location and histological type of RCC. We conclude that RFA presents a safe treatment choice for patients with RCC if long term follow up is maintained.
PMCID: PMC4387349  PMID: 25918603
radiofrequency ablation; nephrectomy; renal cell carcinoma
9.  Whole Exome Sequencing of a Patient with Metastatic Hidradenocarcinoma and Review of the Literature 
Rare Tumors  2015;7(1):5719.
Hidradenocarcinoma is a rare malignancy of the sweat glands with only a few cases reported in literature. The management of these tumors is based on the extent of disease with local disease managed with surgical resection. These can tumors carry a high potential of lymphatic and vascular spread and local and distant metastases are not uncommon. Given the rarity of the tumor and lack of genetic and clinical data about these tumors, there is no consensus on the proper management of metastatic disease. Here in we report the first case of metastatic hidradenocarcinoma with detailed molecular profiling including whole exome sequencing. We identified mutations in multiple genes including two that are potentially targetable: PTCH1 and TCF7L1. Further work is necessary to not only confirm the presence of these mutations but also to confirm the clinical significance.
PMCID: PMC4387361  PMID: 25918615
whole genome exome sequencing; hidradenocarcinoma; mutation sequencing
10.  Retroperitoneal Schwannomas of Renal and Pararenal Origin: Presentation of Two Case Reports 
Rare Tumors  2015;7(1):5616.
Retroperitoneal schwannomas are a rare entity. They originate from the Schwann cells of the nerve sheaths and may be of renal or pararenal origin. We report on two patients with retroperitoneal schwannomas, who received surgery under the suspicion of renal cell carcinoma.
PMCID: PMC4387351  PMID: 25918605
nerve sheath tumor; renal tumor; retroperitoneal tumor; schwannoma
11.  Cherubism in Sub-Saharan Africa: A First Case-Report in a Child 
Rare Tumors  2015;7(1):5675.
Cherubism is rare disease and has been rarely reported in African pediatric population. We report here the case of a 10-year-old child who was referred to our hospital for bilateral jaws swelling. Physical examination revealed bilateral swelling symmetry of the face. Histopathological examination of the biopsy specimen showed loose fibrous stroma, proliferating fibrous connective with tissue interspersed with multinucleated giant cells, small thin walled blood vessels and scattered sparse mononuclear inflammatory infiltrate. Our patient presented cherubism. Cherubism is rarely described in children living in sub-Saharan Africa. Genetic and molecular investigations plays an important role in diagnosis but were not available in poor resources settings in developing countries such as the Democratic Republic of Congo.
PMCID: PMC4387356  PMID: 25918610
cherubism; jaw involvement; child; Kinshasa; Democratic Republic of Congo
12.  Estimation of Daily Proteinuria in Patients with Amyloidosis by Using the Protein-To-Creatinine ratio in Random Urine Samples 
Rare Tumors  2015;7(1):5686.
Measurement of daily proteinuria in patients with amyloidosis is recommended at the time of diagnosis for assessing renal involvement, and for monitoring disease activity. Renal involvement is usually defined by proteinuria >500 mg/day. We evaluated the accuracy of the random urine protein-to-creatinine ratio (Pr/Cr) in predicting 24 hour proteinuria in patient with amyloidosis. We compared results of random urine Pr/Cr ratio and concomitant 24-hour urine collections in 44 patients with amyloidosis. We found a strong correlation (Spearman’s ρ=0.874) between the Pr/Cr ratio and the 24 hour urine protein excretion. For predicting renal involvement, the optimal cut-off point of the Pr/Cr ratio was 715 mg/g. The sensitivity and specificity for this point were 91.8% and 95.5%, respectively, and the area under the curve value was 97.4%. We conclude that the random urine Pr/Cr ratio could be useful in the screening of renal involvement in patients with amyloidosis. If validated in a prospective study, the random urine Pr/Cr ratio could replace the 24 hour urine collection for the assessment of daily proteinuria and presence of nephrotic syndrome in patients with amyloidosis.
PMCID: PMC4387359  PMID: 25918613
kidney; daily proteinuria; albumin; renal insufficiency; 24-hour collection
13.  Paraneoplastic Autoimmune Hemolytic Anemia in Ovarian Cancer: A Marker of Disease Activity 
Rare Tumors  2015;7(1):5598.
Autoimmune hemolytic anemia (AIHA) is a rare paraneoplastic syndrome associated with ovarian malignancies. We report a case of a 77 year-old female with metastatic ovarian carcinoma who presented with worsening anemia from her baseline, and was found to have a warm autoimmune hemolytic anemia. We performed a literature review and analyzed all 10 cases (including our patient) that have been reported to date, and incorporated the clinical presentation, histology and stage of underlying malignancies, types, treatment, prognosis and mechanisms of AIHA in ovarian carcinoma.
PMCID: PMC4387350  PMID: 25918604
autoimmune hemolytic anemia; ovarian cancer; paraneoplastic syndrome
14.  Extraskeletal Myxoid Chondrosarcoma Presenting as an Intradural Spinal Mass: Report of a Rare Clinical Presentation With an Emphasis on Differential Diagnostic Considerations 
Rare Tumors  2014;6(4):5586.
Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm that occurs predominantly in the soft tissues of the lower extremities. Herein we present a case of a 29 year old male who presented with bilateral femoral numbness believed to be the result of prior injury to his back. A magnetic resonance imaging revealed a mass in the T4-T5 epidural space compressing the spinal cord. Laminectomy was performed and the lesion removed piecemeal. The pathology specimen consisted of multiple fragments of dura involved by a myxoid neoplasm with a nodular growth pattern. The tumor cells were arranged in anastomosing cords and strands. Individual tumor cells were small, of uniform size and shape, with small hyperchromatic nuclei and scant eosinophilic cytoplasm. Immunohistochemical stains were performed which showed the tumor cells were diffusely positive for vimentin and focally positive for EMA, S-100 protein and cytokeratin, whereas they were negative for CD34 and CD99. Fluorescence in situ hybridization (FISH) studies showed a clonal population of cells with re-arrangement of the EWSR1 locus, confirming the histologic impression of extraskeletal myxoid chondrosarcoma. This is the first report of a case of an extraskeletal myxoid chondrosarcoma arising from the dura, confirmed to have rearrangement of the EWSR1 gene by FISH. There have only been two other cases of dural based extraskeletal myxoid chondrosarcoma reported prior to our case. We also briefly review the published literature and discuss differential diagnostic considerations for this rare tumor.
PMCID: PMC4274445  PMID: 25568751
extraskeletal myxoid chondrosarcoma; dura; FISH; spine
15.  Knee Glomangioma: A Rare Location for a Glomus Tumor 
Rare Tumors  2014;6(4):5588.
Glomus tumor is a rare, benign neoplasm rising from the glomus apparatus of the skin. It occurs most frequently on fingers and toes and accounts for 1.6% of all soft tissue tumors. Clinical diagnosis may prove difficult if the tumor occurs on an extra digital location. We report a case of a vascular-type glomus tumor (glomangioma) found in an atypical location, namely the lateral aspect of the knee joint.
PMCID: PMC4274446  PMID: 25568752
glomangioma; glomus tumour; knee; extradigital
16.  Prostatic Stromal Sarcoma in an Adolescent: The Role of Chemotherapy 
Rare Tumors  2014;6(4):5607.
Prostatic stromal sarcoma (PSS) is a rare tumor that normally occurs in adult age. Its management relies mainly on surgery. We report the first case of PSS occurring in an adolescent. There was evidence of a good response to chemotherapy including ifosfamide, doxorubicin, vincristine and actinomycin-D, although the final outcome was dismal. A review of the English literature revealed 14 additional patients with PSS treated with chemotherapy: tumor shrinkage was reported in 4 of the 6 evaluable patients. Patients with PSS may benefit from the use of chemotherapy in combination with early aggressive local treatment.
PMCID: PMC4274447  PMID: 25568753
stromal sarcoma; specialized prostatic stroma; chemotherapy; prostatic neoplasm
17.  Spontaneous Rupture of the Kidney Affected by Multifocal Papillary Renal Cell Carcinoma 
Rare Tumors  2014;6(4):5568.
Papillary renal cell carcinoma (pRCC) represents the second most common type of malignant renal epithelial tumor (represents the 10% of the kidney’s carcinoma) and can be subclassified in the basophile type I and eosinophile type II. We report a clinical case of spontaneous rupture of the kidney affected by multifocal (42 tumors foci) pRCC in a young man 53 years old, without showing earlier specific cancer signs and symptoms. Prognosis for type I pRCC is better than type II pRCC, but it is anyway related to the tumoral grade, to the tumoral stage and to the diagnostic precocity. Signs and symptoms are very similar to those characterizing the more frequent clear cell carcinoma. Nevertheless in the 40% of the cases the lesion is asymptomatic. To our knowledge, this is the first case of spontaneous rupture of the kidney affected by multifocal pRCC in literature without showing earlier specific cancer signs and symptoms.
PMCID: PMC4274443  PMID: 25568749
papillary renal cell carcinoma; multifocality; prognostic factor; rupture of kidney
18.  Malignant Peripheral Nerve Sheath Tumor of the Anterior Mediastinum: A Rare Presentation 
Rare Tumors  2014;6(4):5528.
Malignant peripheral nerve sheath tumor (MPNST) is a rare tumor that accounts for 5% of all thoracic neoplasm usually located in the posterior mediastinum and is generally associated with a poor outcome. We present a case of MPNST of the anterior mediastinum presenting in a rare location leading to diagnostic dilemmas and treated primarily by surgical resection.
PMCID: PMC4274440  PMID: 25568746
malignant peripheral nerve sheath tumor; anterior mediastinum; immunohistochemistry
19.  Extranodal Rosai-Dorfman Disease of the Bone Treated with Surgery and Radiotherapy 
Rare Tumors  2014;6(4):5531.
Rosai-Dorfman disease (RDD) is a rare, benign histiocyte disorder originally described by Rosai and Dorfman in 1969 as sinus histiocytosis with massive lymphadenopathy. It most typically presents as massive, painless cervical lymphadenopathy, and it is most often found in adolescents and young adults. Extranodal involvement is a common feature of RDD and may occur in more than 40% of patients. Less commonly, the disease can be seen in the bone. There are scattered case reports discussing the use of radiotherapy in the treatment of RDD. Here, we present a case of extranodal RDD of the femur in a 49-year-old African American female. The patient underwent three surgical procedures prior to undergoing radiotherapy. Pathology was consistent with extranodal RDD. The cells stained positive for CD68 and S-100. Pathognomonic features such as emperipolesis and replacement of the bone marrow by diffuse infiltrating of histiocytes and intervening bands of plasma cells proliferation were noted. Prior to radiotherapy, the patient required a walker to assist with ambulation and was utilizing a wheelchair while in the clinic. The decision was made to proceed with 30 Gy of external beam radiotherapy in 15 fractions. After five fractions, the patient’s pain resolved entirely. She no longer required pain medication and was ambulating without assistance. She experienced no adverse events from the radiation. Extranodal RDD is a rare disorder, and evidence for treatment is derived from scattered case reports. Previous reports have indicated a dose response to radiotherapy in the 20-30 Gy range for RDD; however, our patient developed complete resolution of her symptoms after 10 Gy. While the optimal dose regimen has not yet been established, symptomatic patients appear to benefit from external beam radiotherapy for extranodal RDD.
PMCID: PMC4274442  PMID: 25568748
Rosai-Dorfman disease; radiotherapy; histiocyte; dose regimen
20.  Interdigitating Dendritic Cell Sarcoma Presenting in the Skin: Diagnosis and the Role of Surgical Resection, Chemotherapy and Radiotherapy in Management 
Rare Tumors  2014;6(4):5573.
We report the case of an interdigitating dendritic cell sarcoma (IDCS) presenting in the skin. A 41-year old woman had a slowly enlarging mass on her right scapula that was excised multiple times under a presumptive diagnosis of a recurrent sebaceous cyst. However, the lesion was refractory to standard therapies. History and physical exam was unrevealing for any systemic signs or symptoms of disease. The patient’s metastatic work-up was negative. The lesion was resected with wide margins and was found to be consistent with IDCS. Patients that present with IDCS on the skin may present concurrently with metastatic disease and may have increased risk of secondary malignancies. The use of adjuvant chemoradiation after primary resection is controversial. However, the use of chemoradiation likely has benefit for local regional control for primary tumors that are unamendable to complete primary resection.
PMCID: PMC4274444  PMID: 25568750
interdigitating dendritic cell sarcoma; hematolymphoid neoplasm; extranodal disease; radiation therapy; chemotherapy
21.  Response to Targeted Therapy in Urachal Adenocarcinoma 
Rare Tumors  2014;6(4):5529.
We report the case of a young woman diagnosed with metastatic urachal carcinoma. A multimodal approach was used for the management of this patient. Due to disease progression despite surgery and two different chemotherapy regimens (neoadjuvant capecitabine + irinotecan + oxaliplatin and docetaxel + cisplatin after surgery), treatment with sunitinib was eventually started. Treatment with sunitinib resulted in stable disease and improvement of symptoms. Sunitinib was discontinued due to the occurrence of metrorrhagia, and restarted one week later. Disease eventually progressed and the patient died 18 months after the onset of symptoms. This is the first report on the use of sunitinib for the management of urachal carcinoma and provides initial evidence supporting the use of targeted therapy in this setting.
PMCID: PMC4274441  PMID: 25568747
sunitinib; targeted therapies; urachal adenocarcinoma
22.  Mucinous Urothelial Carcinoma of the Renal Pelvis 
Rare Tumors  2014;6(4):5485.
Urothelial carcinoma with abundant myxoid stroma is a newly-described and extremely rare entity. Since only very few cases have been reported, there is no consensus on its nomenclature. Microscopic examination revealed invasive urothelial carcinoma with widespread low-grade noninvasive areas. There were focal invasive areas in the neighborhood of the renal parenchyma. Malignant urothelial tumor/cell groups localized in the stroma had abundant myxoid/mucinous background in the invasive areas. The cytoplasm of the tumoral cells was more eosinophilic in these areas and the cells formed small groups and cords. Histochemically, PAS and Alcian Blue were positive in the cytoplasm of the tumoral cells and in the stroma while negative in the non-mucinous areas. Immunohistochemically, the tumoral cells of the mucinous invasive areas diffusely expressed MUC1 and MUC2. We discuss the origin of the mucinous/myxoid stroma, the tumor’s nature and its nomenclature with histochemical and immunohistochemical features.
PMCID: PMC4274439  PMID: 25568745
mucinous; myxoid; urothelial carcinoma; renal pelvis; MUCs
23.  Primary Gastric Burkitt’s Lymphoma 
Rare Tumors  2014;6(4):5300.
The primary gastrointestinal non-Hodgkin’s lymphoma, although rare, is among the most common extra-nodal lymphomas, considering that gastric lymphomas are more common than intestinal lymphomas. Burkitt’s lymphoma (BL) is an aggressive form of B-cell lymphoma that is typically endemic in Africa, while non-endemic cases are found in the rest of the world. Primary gastric BL is extremely rare and only around 50 cases have been reported worldwide. Here we present the case of a young HIV-negative male, who was referred to our department with a stage IV gastric BL. He was planned for palliative chemotherapy, but after the first cycle of chemotherapy he succumbed to the progression of the disease.
PMCID: PMC4274437  PMID: 25568743
Burkitt’s lymphoma; gastric; radiation oncology; medical oncology
24.  Blastic Plasmacytoid Dendritic Cell Neoplasm with Extensive Cutaneous and Central Nervous System Involvement 
Rare Tumors  2014;6(4):5474.
Blastic plasmacytoid dendritic neoplasm is an exceedingly rare tumor that has undergone several changes in nomenclature over the last two decades, largely because of confusion regarding its cell of origin. It does, however, have distinctive clinical features with a particularly aggressive clinical course and no standard treatment. Overall, prognosis is poor and relapse is routine after initial response to chemotherapy. In this report, we describe a typical patient with this disease and reconcile the available literature and its evolution. We emphasize the leukemic nature of this tumor’s behavior, with extensive central nervous system and skin involvement, and describe for the first time a potential role for maintenance chemotherapy in its treatment.
PMCID: PMC4274438  PMID: 25568744
blastic plasmacytoid dendritic cell neoplasm; skin; central nervous system; leukemia
25.  Squamoid Cystosis of Pancreatic Ducts: A Variant of a Newly-Described Cystic Lesion, with Evidence for an Obstructive Etiology 
Rare Tumors  2014;6(3):5286.
We describe a 40-year-old man who was found to have a cystic mass in the pancreatic tail during workup for weight loss and abdominal discomfort. Although computed tomography scan showed a single cyst associated with dilatation of the main pancreatic duct, gross and histologic examination of the distal pancreatectomy specimen actually revealed a central cyst that was surrounded by multiple smaller cystic spaces. This distinctive appearance was formed from extensive cystic dilatation and squamous metaplasia of the native pancreatic duct system. Further, a traumatic neuroma was discovered near the junction between normal and abnormal parenchyma. We believe that this case represents a variant of the newly-described squamoid cyst of pancreatic ducts which we term squamoid cystosis of pancreatic ducts. The presence of chronic pancreatitis and a traumatic neuroma supports the hypothesis that squamoid cysts are non-neoplastic lesions arising from prior duct obstruction.
PMCID: PMC4178271  PMID: 25276318
pancreas; tumors; benign

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