Intradural disc herniation of L2/3: A case report and literature review.

Background: Intradural herniation (IDH) or transdural disc herniation is a rare presentation of lumbar disc disease. Preoperative imaging findings should be carefully and thoroughly interpreted. Although imaging modalities such as computed tomography (CT) or magnetic resonance imaging (MRI) are readily available, a definitive diagnosis cannot be made based solely on these modalities. Operative procedures must be planned to prevent unexpected complications. Case description: A 67-year-old man presented with right lower extremity weakness and numbness with bowel and bladder involvement for 2 weeks, after falling from a standing position. MRI revealed a large herniated disc at L2-L3, which was suspected to be IDH. Posterior discectomy and interbody fusion were also performed. Intraoperative findings revealed no disc material in the epidural space or dural sac tenting. Dorsal midline durotomy was performed, and a mass-like lesion was found and resected. Subsequently, pathological analysis revealed disc tissue with evidence of moderate chronic inflammation and a focal increase in fibrosis. The patient was discharged without complications. Results Outcome: Lower extremity strength improved to grades IV-V, accompanied by a return to normal bowel and bladder function within 1 month, without any wound complications. Lower extremity strength recovered fully to grade V, and the patient started walking independently within 6 months. Conclusions: A large disc herniation, suspected to be an IDH, should be thoroughly investigated by carefully reviewing MRI scans before proceeding with any surgical procedure to prevent unexpected situations. Nonetheless, preoperative imaging alone does not ensure a definitive diagnosis, and the differential diagnosis must include other mass-like lesions. Intraoperative findings and pathological reports are essential for definitive diagnosis of IDH.

Extremely high alpha-fetoprotein-producing adrenal hepatoid adenocarcinoma.

Hepatoid adenocarcinoma (HAC) is a rare tumour that produces an alpha-fetoprotein (AFP) mimicking hepatocellular carcinoma (HCC). Adrenal HAC is exceedingly rare. Here we report extremely high AFP-producing adrenal HAC, the first case in Thailand. A 47-year-old man presented with left flank pain and weight loss for 2 months. A palpably huge left flank mass was observed on physical examination. CT revealed a 7 cm enhanced mass involving the left adrenal gland and multiple contrast-enhanced hypodense masses in both liver lobes. The largest was a 3.7 cm at liver segment-VII without cirrhotic background, with an AFP level of 321 495 ng/mL. Both adrenal and liver biopsies were performed. This patient received a diagnosis of advanced adrenal HAC. Unfortunately, the tumour progressed, causing massive upper gastrointestinal bleeding and death. Adrenal HAC is challenging to diagnose, which multifocal HCC, pheochromocytoma and adrenocortical carcinoma should be excluded. Surgical resection is preferred among resectable patients. However, no systemic therapy has been standardised.

Clinical Characteristics and Prognostic Factors in Pediatric Vascular Tumors.

BACKGROUND: The most common type of vascular tumors reported among children is hemangioma. The determinant factors to predict clinical outcomes among those patients were not well studied. OBJECTIVE: The study aimed to explore clinical characteristics and treatment approaches as well as associated prognostic factors of vascular tumors specifically in a pediatric population. METHODS: Pediatric patients with a confirmed diagnosis of vascular tumors between January 1, 2005 and December 31, 2017 were enrolled in this study. Clinical data includes initial clinical manifestations with associated complications, and diagnostic studies were used. To establish a diagnosis, the treatment modalities provided and final outcomes were retrospectively reviewed and analyzed. RESULTS: In all, 50 patients with a confirmed diagnosis of vascular tumors were enrolled. The median age at diagnosis was 11.5 years with equal gender distribution. The most common type of vascular tumor was hemangioma (n=41, 82%), followed by pyogenic granuloma (n=4, 8%), kapasiform hemangioendothelioma with Kasabach-Merritt phenomenon (n=2, 4%), infantile hepatic hemangioma (n=2, 4%), and juvenile nasal angiofibroma (n=1, 2%). The median age at diagnosis among patients with cutaneous vascular tumors (12.4 years) was significantly older than the age of those with visceral vascular tumors (1.3 years) witha P-value of 0.009. The mean size among patients with visceral tumors (7.46±4.84 cm) was significantly greater than the size among patients with cutaneous tumors (3.21±3.7 cm) with a P-value of 0.023. The size of the tumor was the only independent risk factor associated with clinical outcomes. CONCLUSION: Age at diagnosis in cutaneous vascular tumors was significantly older than in visceral vascular tumors. Clinical outcomes are favorable among most patients and the size of the tumors is an independent risk factor associated with outcomes.

Characteristics of Marjolin's Ulcers in 21st Century: A Retrospective Study, Systematic Review, and Surgical Guideline Recommendation.

Marjolin's ulcer is rare and aggressive malignancy. The objective was to conduct a review of Marjolin's ulcer cases to gain a better understanding and its management. A retrospective study of patients with Marjolin's ulcers at our hospital between 2010 and 2019 was conducted. For the systematic review, the electronic database of the National Library of Medicine was searched and articles published between 2000 and 2019 were included. A total of 14 patients were included in the retrospective study. Twelve patients were male. The mean age was 59.71 years. The latency period was 27.78 years. The most common malignancy was squamous cell carcinoma, and 71.43% had developed on the lower extremities. Well-differentiated squamous cell carcinomas occurred in 38.46% of the cases Rates of lymphovascular and perineural invasion were 30.77 and 23.08%, respectively. Results from the literature search yielded 80 case reports (91 cases) and 42 retrospective reviews (1216 cases). Most cases were male and the most common location was lower extremities. The latency period for the acute and chronic periods was 2.75 months and 20.75 years, respectively. The most common malignancy was squamous cell carcinoma (92.27% in the retrospective reviews) and 59.98% of the cases exhibited well-differentiation. Rates of regional lymph node involvement and distant metastases were 10.20 and 12.09%, respectively. Marjolin's ulcer in 21st century is not so rare. The most common is well differentiated squamous cell carcinoma. Early flap surgery or skin grafts for chronic ulcers or burn scars are recommended to reduce risk. Guideline for surgical management is established.

Osteoradionecrosis of the Chest Wall.

We present the case of a 48-year-old woman with a chronic ulcer with bony erosion over the right chest wall who had undergone adjuvant chemoradiotherapy for right breast cancer 18 years previously. Preoperative computed tomography revealed a large soft tissue ulcerative lesion with bony destruction of the anterolateral aspect of the right fifth and sixth ribs. Biopsy showed no evidence of recurrent malignancy. En bloc resection including the necrotic ulcer and partial resection of the fourth to sixth ribs was performed, resulting in a chest wall defect that exposed the right diaphragm and the right, middle, and lower lobes of the lung. Synthetic mesh was used to reconstruct the rib defect and prevent paradoxical respiration. A pedicle transverse rectus abdominis musculocutaneous flap was used for soft tissue and skin reconstruction.

Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma with Intramuscular Lipoma-Like Component of the Thigh.

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS) is a locally aggressive mesenchymal neoplasm composed either entirely or partly of an adipocytic proliferation showing at least focal nuclear atypia in both adipocytes and stromal cells. ALT most frequently occurs in deep soft tissue of proximal extremities (thigh and buttock) and usually presents as a deep-seated, painless mass that can slowly attain a very large size, which is one of the most common sarcomas of extremity. In the patients who presented with deep-seated tumor, distinction between intramuscular lipoma and well-differentiated liposarcoma is important due to the differences in treatment and prognosis. However, atypical well-differentiated liposarcoma with intramuscular lipoma-like component of the thigh is extremely rare. Moreover, the infiltrative growth pattern or intramuscular component may lead to a misinterpretation as intramuscular lipoma on a small biopsy. We present an unusual case of a female who presented with symptomatic mass at the thigh which has rarely been reported in English literature as an infiltrative intramuscular lipoma-like growth pattern of well-differentiated liposarcoma. Therefore, preoperative diagnosis is necessary, and correlation with imaging studies is required when one encounters with a large deep-seated mass. Atypical lipomatous tumor or well-differentiated liposarcoma should be kept in mind in the patient who presents with abnormal thigh mass.

Unusual Case of Concurrent Retroperitoneal Congenital Infantile Fibrosarcoma and Cellular Type Congenital Mesoblastic Nephroma.

BACKGROUND: Although congenital infantile fibrosarcoma (cIFS) is a rare soft tissue sarcoma among children, it constitutes one of the most common soft tissue sarcomas during the first year of life. Congenital mesoblastic nephroma (CMN) is the most common benign renal tumor usually developing during the first 3 months of life. cIFS and cellular type CMN (cCMN) share not only similar histopathologic features but identical molecular genetic abnormality including the ETV6/NTRK3 fusion gene. Here, we report an unusual case of cIFS occurring with cCMN. CASE PRESENTATION: An 18-month-old girl presented with a 1-month history of abdominal distension and a few days' history of a palpable abdominal mass. A large heterogenous mass sized 9.0×11.2×11.6 cm on the right side of the abdomen and an isolated heterogenous lesion sized 4×4.5 cm within the right kidney were noted from the imaging study. Pathologic findings were consistent with cIFS and cCMN of the right kidney. In addition, both pathologic specimens contained the ETV6/NTRK3 fusion gene. CONCLUSION: Although cIFS and cCMN share similar histopathologic features and molecular genetic abnormality, simultaneous occurrence of these 2 types of tumor is exceedingly rare. To our knowledge, this is the first unusual case report of concurrent cIFS and cCMN.

Primary angiosarcoma of the breast presenting with a benign vascular skin-like lesion and expanding hematoma: a case report of an extremely rare tumor.

Primary breast angiosarcoma is an extremely rare tumor type (0.05% of primary breast cancers) for which diagnosis can be difficult. They arise within the breast parenchyma and typically present as a breast mass. Here, we present the case of a 30-year-old female with spontaneous hypervascular skin on her right breast with progressive enlargement presenting as an expanding hematoma. A chest computed tomography revealed a hypervascular mass in the right breast abutting the pectoralis muscle and cystic changes within the mass. A core needle biopsy revealed an angiosarcoma. In this case study, we report a patient who, with no history of any known risk factors, presented with a benign vascular skin lesion as the first sign of angiosarcoma followed by an expanding hematoma, which is an extremely rare manifestation of the disease. Microscopic examination demonstrated a low-grade angiosarcoma on the skin, while a high-grade angiosarcoma was found in the breast parenchyma.

Cholangiocarcinoma in a Child with Progressive Abdominal Distension and Secondary Hypercalcemia.

Cholangiocarcinoma is extremely rare in childhood and has been reported in association with other underlying diseases. The survival and prognosis are dismal especially in patients with unresectable or advanced stage cholangiocarcinoma. Overall survival in patients with metastatic cholangiocarcinoma could be increased by using combination chemotherapy with cisplatin and gemcitabine. A case of childhood cholangiocarcinoma was hereby reported.

An Unusual Presentation of Idiopathic Lymphatic Cyst of the Thigh.

Lymphatic cyst is a collection of lymphatic fluid with an epithelial lining which can either represent lymphatic malformations or may occur after trauma or operation. Idiopathic lymphatic cyst of the thigh in adulthood is extremely rare. We report a case of a 27-year-old Thai female presented with a rapidly growing mass over her right thigh. The magnetic resonance imaging scan demonstrated a well-defined, thin rim, enhancing simple cyst. The cyst disappeared for a few days after aspiration. Completed surgical resection was performed for definitive diagnosis and treatment. Immunohistochemical studies revealed the cell lining was positive for CD34, CD31, and D2-40 and negative for calretinin. To our best knowledge, idiopathic lymphatic cyst of the thigh in young adult has never been reported and should be kept in mind in the patient who presents with abnormal thigh mass.

Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener's granulomatosis).

The purpose of this study was to describe a case of necrobiotic xanthogranuloma scleritis in a 53-year-old male with unilateral progressive visual loss, scleritis, prolonged fever, and multiple mononeuropathy. Scleral biopsy showed necrosis with small abscess, and the pathological tissues revealed submucosal infiltration of mononucleated foamy histiocytes (xanthoma cells), hemosiderin-laden macrophages, neutrophils, lymphocytes, plasma cells, and erythrocytes without Touton giant cells or cholesterol clefts. Serum protein electrophoresis showed polyclonal gammopathy. All infectious investigations were negative. Afterward, this patient was diagnosed with granulomatosis with polyangiitis based on granuloma found in scleral tissue, vasculitis seen in sural nerve biopsy and positive serologies (C-ANCA and anti-PR3 antibody). He was treated with high-dose corticosteroid and later with intravenous cyclophosphamide monthly. He responded well to treatment, both eye and systemic conditions. Necrobiotic xanthogranuloma scleritis could be an early presentation of granulomatosis with polyangiitis.

Alveolar soft part sarcoma of flexure tendon.

Alveolar soft part sarcoma (ASPS) is a very rare sarcoma, report <1% of all soft tissue tumor. Majority of cases were young adult and tumor occurred in lower extremities and trunk. Here, we present a case of 53-year-old Thai female with rapidly glowing mass over her right forearm. The magnetic resonance imaging scan demonstrated a hypervascular mass with multiple feeding vessels located in flexure tendon of right forearm. Ultrasound-guided biopsy revealed malignant poorly differentiated epithelioid neoplasm with clear cell feature and focal necrosis. Surgery may be considered the first-line treatment in localized ASPS and may potentially increase long-term survival. Complete surgical excision is the mainstay of treatment. TFE3 and Cathepsin K immunohistochemistry are useful in confirming a diagnosis of ASPS with a distinctive clinicopathologic features.

An experimental study of the effect of pre-operative administration of cilostazol on random skin flap survival in rats: double blinded randomized controlled trial.

BACKGROUND: Insufficient arterial blood flow is the one cause of flap necrosis. Cilostazol is an inhibitor of phosphodiesterase III and increases cyclic AMP level in vascular smooth muscle cell causing vasodilation. Therefore, effect of cilostazol is expected to improve the viability of the flap. METHODS: Double blinded randomized controlled trial was conducted. The study was to compare the survival of dorsal rat flaps between preoperative cilostazol supplemented diet and regular diet. The flap survival area was measured using PixArea Image software on post operative day 1,3,5 and 7. Fluorescein injection was performed to evaluate the exactly area of flap survival on postoperative day 7 and morphology of arterioles and venules were examined by histopathologic examination. RESULTS: A statistical significance was found in the percentage of area of flap survival between cilostazol supplemented diet and control group on postoperative day 3, 5 and 7 (p < 0.05). Fluorescein injection showed the higher area of flap survival in cilostazol group than the control group (p < 0.05). Histopathologic examination showed dilation of vessels in the cilostazol group. CONCLUSION: Preoperative cilostazol in rats can enhance skin flap survival.

Meningeal hemangiopericytoma only diagnosed at the time of late bone metastasis.

Hemangiopericytoma is a rare neoplasm. Primary meningeal hemangiopericytomas account for 1 to 7% of all meningeal tumors. In the literature, meningeal hemangiopericytomas are mainly case reports, which confirm an aggressive behavior with a high rate of local recurrence and extracranial metastasis. Metastasis can be seen many years after initial surgical excision of the primary tumor, and the most common sites include the bone, liver and lung. We present a pathological study of four meningeal hemangiopericytomas with bone metastases. All patients are male with a mean age of 46.5 years. Metastases only involved bone. Three out of four lesions were initially misdiagnosed as meningiomas. Only one case was initially correctly diagnosed as meningeal hemangiopericytoma. All patients underwent surgery with complete resection. Only the patient immediately diagnosed with meningeal hemangiopericytoma received postoperative radiation therapy. Three patients had bone metastases without local recurrence including the one who received radiation therapy. One patient recurred locally after 7 years, and bone metastasis was found at the same time. Our cases confirm that meningeal hemangiopericytomas are a separate entity and have a high recurrence rate despite complete surgical resection, with extracranial metastases, mainly to bone, even after long intervals.

Clinicopathological study of primary superficial leiomyosarcomas.

BACKGROUND: Primary superficial leiomyosarcomas (PSL) are rare malignant lesions that are subdivided into cutaneous and subcutaneous tumors. Primary cutaneous and subcutaneous leiomyosarcomas differ not only as to primary site of origins, but also to differences in prognosis. Guidelines for management and follow-up are not clearly defined in the literature. MATERIAL AND METHOD: Retrospective review was conducted from the patient's chart between January 2000 and December 2009. Histopathology, immunohistochemistry, and clinical and surgical records were reviewed. RESULTS: The authors found five cases of PSL and divided them into two cases of cutaneous leiomyosarcomas and three cases ofsubcutaneous leiomyosarcomas. Overall, mean age of the patients was 42.4 years, male: female ratio was 4:1. Clinical presentations were painless mass. Wide excisions were performed in three cases with 2 cm margins. No local recurrence was found in the period of follow-up (6 months to 3 years). One case presented with bony metastasis five years after operation. CONCLUSION: PSL are rare tumors. Surgical resection remains the main option for curative treatment. Wide excision with at least 2 cm peripheral margins and a depth that includes subcutaneous tissue and fascia are recommended. The natural history of these tumors is not clearly defined. All patients should be followed-up for a period of at least five years after treatments. The authors hoped that further study of these tumors would result in better treatments and follow-up guidelines to be a benefit to such patients in the future.

A Chronic Myelogenous Leukemia (CML) Case with a Cryptic Insertion of the ABL1 Gene of Chromosome 9 into 22 Resulting in a Fusion Signal on the Derivative 22: 46,XY.ish ins(22;9)(q11.2;q34q34)BCR+,ABL1.

Chronic myelogenous leukemia (CML) is characterized by the specific cytogenetic translocation t(9;22)(q34;q11.2), also called the Philadelphia (Ph) chromosome. We present a case of a cryptic BCR/ABL1 fusion, which was not originally detected by standard karyotyping. The patient is a forty-seven-year-old man who presented with leukocytosis. Bone marrow biopsy was consistent with CML in chronic phase with no increase in myeloblasts. Conventional cytogenetic studies revealed a 46,XY karyotype. Despite this finding, the patient was started on hydroxyurea therapy followed by Gleevec. At six-month follow-up, a repeat karyotype was again normal, though FISH analysis was positive for BCR/ABL1 fusion. FISH performed on previously G-banded metaphases showed a very rare cryptic insertion involving 22q11. While most genetic abnormalities in CML can be diagnosed using classical cytogenetics, molecular studies remain the gold standard in definitively identifying the characteristic BCR/ALB1 fusion. This case represents one of the variant cryptic rearrangements in CML where clinical correlation with morphologic, immunophenotypic, cytogenetics and FISH findings are indicated and highlights the importance of molecular testing at the time of primary diagnosis.

Malignant proliferating trichilemmal tumors with CD34 expression.

Malignant proliferating trichilemmal tumors (MPTT) are rare neoplasm arising from outer root sheath of hair follicle, the diagnosis of which is base essentially on histological features resulting in occasionally misdiagnosis of squamous cell carcinoma. In difficult cases, however evaluation of additional parameters may be needed to differentiate benign proliferating trichilemmal tumor from MPTT or differentiate PTT and MPTT from squamous cell carcinoma. We report two cases of MPTT on which, in addition to histologic features, we have determined p53 immunohistochemical expression pattern, proliferative fraction, and CD34 expression. For comparison, concurrent proliferating trichilemmal tumors (PTT) and trichilemmal cysts (TC) as well as well-differentiated squamous cell carcinoma (SCC) were studied. The two MPTTs show expression of p53 with increased proliferative index as well as all three SCC. The PTTs and TCs stained negative and few basal cells for p53 and Ki-67, respectively. MPTTs exhibit CD34 immunoreactivity, indicating trichilemmal differentiation. The contrast p53 and Ki-67 expression pattern in MPTT and PTT may be helpful in the diagnosis of MPTT. Expression of CD34 may be an additional feature to distinguish MPTT from SCC.