[Bone marrow serous degeneration complicating acute leukemia]. / Transformation gélatineuse de la moelle osseuse au cours d'une leucémie aiguë.

INTRODUCTION: Serous degeneration of bone marrow is a rare hematological complication, of multiple etiologies, which physiopathology is yet to be fully understood. It is described as a focal hypoplasia of the bone marrow and atrophy of its adipocytes coupled with an extracellular deposit of an eosinophilic substance that corresponds to "hyaluronic acid". The prognosis of these lesions depends on the etiological diagnosis. OBSERVATION: We report the case of a 19-year-old female patient without no notable pathological history, hospitalized in a regional hospital for mucocutaneous pallor with an impaired general condition. The biological assessment revealed a pancytopenia, and the presence of 60 % blast cells on the blood smear, which was in favor of acute leukemia. As part of the etiological assessment, a bone marrow biopsy was performed, revealing a typical aspect of serous degeneration. The bone marrow cellularity was reduced, made of rare hematopoietic cells, dissociated by pale extracellular deposits that were colored by PAS and alcian blue, with no individualized blasts. DISCUSSION: Serous transformation of the hematopoietic marrow is a rare condition, described at the end of the 19th century. The most severe forms are observed in young patients with hypoplasia. It most often manifests as pancytopenia at the beginning, if not then by an isolated cytopenia, anemia or thrombocytopenia. It is interesting to underline the contribution of MRI in diagnosis. The foci of bone marrow serous degeneration are characterized in the long bones by a fluid signal with a T1 hypo-signal and a T2 hyper-signal with no enhancement after injection of gadolinium, unlike bone metastases. The histology of bone marrow biopsy finds focal marrow hypoplasia of the hematopoietic lines associated with a depletion of adipocytes and an eosinophilic gelatinous interstitial infiltration alcian blue and PAS positive. The histological differential diagnosis is amyloidosis due to red Congo positivity, a previous biopsy (the absence of adipose tissue or hematopoietic tissue with granulation tissue and bone formation) and interstitial edema. Bone marrow necrosis can also be observed. Serous degeneration of bone marrow can be a symptom of a chronic systemic illness. In most cases, it is found in cases of severe chronic malnutrition, such as anorexia nervosa, certain cancers (acute leukemia), systemic diseases (lupus) or severe infection. Though there is no specific treatment for this complication, the treatment of the cause most often allows for the complete regression of the peripheral and spinal anomalies. CONCLUSION: In rare cases, as is the case in our observation, the serous degeneration of bone marrow can reveal underlying solid tumors or hemopathies, knowledge of it can help guide the investigations towards these etiologies.

Synchronous colonic adenoma and intestinal marginal zone B-cell lymphoma associated with Crohn's disease: a case report and literature review.

BACKGROUND: Lymphoma and dysplasia are rare complications of long-standing Crohn's disease. We report an exceptional case of a synchronous intestinal marginal zone B-cell lymphoma (MALT lymphoma) and colonic adenoma in a Crohn's disease patient. CASE PRESENTATION: A 50-year-old male patient presented with right lower quadrant for the last 9 months. He also had associated weight loss and diarrhea alternating with constipation. Ileo-colonoscopy revealed a pseudopolypoid appearance of the colonic and ileal mucosa with many discontinuous ulcerations with a 3 cm sessile polypoid mass at 17 cm from the anal verge. Histological examination of the polypoid lesion revealed an adenoma with high grade dysplasia, while the biopsies of colonic mucosa showed histologic features of Crohn's disease. Abdominal computed tomography scan (CT scan) and magnetic resonance imaging (MRI) showed circumferential wall thickening of the colon and ileum, enlarged mesenteric lymph nodes and a sessile polypoid mass of the rectosigmoid junction. The patient was scheduled for an ileocoletectomy with resection of the upper rectum and ileorectostomy. The histological examination of the resected segment showed histologic features of Crohn's disease, a recto-sigmoid polyp with high grade. dysplasia and extensive small lymphocytic infiltrate in both colonic and ileal wall which is strongly stained by CD20 and BCL2. The diagnosis of MALT lymphoma with adenoma on a background of Crohn's disease was made. The patient successfully completed 8 cycles of Rituximab+ chlorambucil chemotherapy. Nowadays the patient is asymptomatic without evidence of lymphoproliferative recurrence 10 months after surgery. CONCLUSION: We report the first case in the literature of Malt lymphoma with colonic adenoma associated with Crohn's disease, and discuss his unique macroscopic and histological features in a patient. Without immunosuppressive therapy.

Unusual Association of Large Cell Neuroendocrine Carcinoma of the Bladder and Prostatic Adenocarcinoma Within a 76-Year-Old Man.

Large cell neuroendocrine carcinoma (LCNEC) is one of the rarest types of bladder cancer, with an aggressive course and a poor prognosis. We report a case of LCNEC of the bladder associated with a prostatic adenocarcinoma. A very rare association, to our knowledge, is described for the first time in the literature. The patient was a 76-year-old non-smoker male, who presented with intermittent hematuria and dysuria. Cystoscopy revealed a lesion on the right lateral bladder wall. Biopsy was in favor of a LCNEC with muscle invasion. The CT scan showed the presence of a second lesion in the prostate, confirmed by histological examination. The patient was treated by neoadjuvant chemotherapy of the cisplatin-etoposide type for large-cell bladder neuroendocrine carcinoma, and hormone therapy with luteinizing hormone-releasing hormone (LH-RH) analogs for prostatic adenocarcinoma, followed by radical surgery. Given the rarity of this tumor, LCNEC treatment lacks precision. Many cases are published with different therapeutic strategies. A literature review would be interesting to codify the therapeutic strategy for this rare tumor.

Synovial Sarcoma of the Submaxillary Salivary Gland: A Rare Location and Challenging Diagnosis.

Synovial sarcoma is a high-grade sarcoma. The periarticular region, deep soft tissues, and the extremities are where it is most frequently found. The head and neck regions are rarely affected and salivary gland localization is rather rare, especially the submaxillary gland. The process of diagnosis and therapeutic management remains challenging, particularly in cases with uncommon tumor locations where the establishment of a universal therapeutic consensus is complicated. Early diagnosis and a multidisciplinary approach can lead to success without locoregional recurrence or distant metastases.

Primary Hepatic Squamous Cell Carcinoma: A Case Report.

Primary squamous cell carcinoma (SCC) of the liver, a notably uncommon type of cancer, is frequently linked with diverse hepatic conditions including hepatic cysts, hepatolithiasis, and hepatic teratoma. Literature indicates that only approximately 30 cases of primary SCC of the liver have been documented. Herein, we report a 54-year-old previously healthy patient who was presented with cholangitis symptoms. Examinations revealed normal vitals. However, deranged liver function with transaminitis and hyperbilirubinemia were noticed. A CT scan showed a hepatic mass with bile duct dilation. Biopsy confirmed hepatic squamous cell carcinoma, leading to chemotherapy treatment. Despite treatment, the survival outcomes for this cancer remain limited, and the prognosis is generally unfavorable.

Pre-sternal Embryonic Dermoid Cyst: A Case Report.

Epidermoid and dermoid cysts are benign, usually slow-growing lesions classified as ectodermal inclusion cysts. These cysts form when epithelial remnants become trapped during the closure of the first and second branchial arch; however, a few cases are related to trauma or are iatrogenic. Diagnosis is made based on the cyst development history and imaging such as ultrasound. Surgical excision is the treatment of choice to avoid complications. We report a case report of a dermoid cyst in the pre-sternal region in a 17-year-old male patient.

Leiomyoma: An Exceptional Benign Tumor of the Kidney.

Renal leiomyoma is a benign finding in kidney pathology. It has been documented in various organs; renal location is less frequent and has been rarely documented in the literature. We present here the case of a renal leiomyoma revealed by an abdominal mass and flank pain. The diagnosis of certainty is histological, generally on surgical specimens. Due to the challenges associated with clinically diagnosing this tumor, a high level of suspicion is warranted when a patient presents with sizable and clearly defined renal lesions.

Papillary Neoplasms of the Gallbladder and Extrahepatic Bile Ducts: A Report of Two Cases With Associated Invasive Carcinoma.

Intracholecystic papillary neoplasm (ICPN) of the gallbladder is a macroscopically visible premalignant lesion protruding into the gallbladder lumen, with infrequent association with invasive adenocarcinoma. Intraductal papillary neoplasm of the bile ducts (IPNB) is a non-invasive lesion characterized by intraductal papillary or villous architecture. Both ICPN and IPNB are rare findings in the gallbladder and biliary tract pathology. Diagnosis relies on clinical manifestations, imaging techniques, and comprehensive histological examination. Here, we present two cases: a 63-year-old male with mild abdominal pain found to have a gallbladder mass, diagnosed histologically as ICPN with associated invasive carcinoma; and a 65-year-old female with chronic jaundice and a large tumor mass in the common bile duct, histologically diagnosed as IPNB with associated invasive carcinoma. These cases highlight the importance of a careful and thorough microscopic examination to rule out differential diagnoses and to reveal any potential invasive carcinoma associated with these uncommon lesions.

Giant Poroid Hidradenoma of the Forearm.

Poroid hidradenoma represents an uncommon and benign tumor originating from skin adnexa. It falls under the category of sweet duct neoplasms, along with poromas. It affects the elderly population most frequently. Typically, it emerges as a small, distinct, and painless lump beneath the skin's surface, often occurring on the head and neck regions. It is characterized by a low risk of malignant transformation. Accurate identification relies especially on histomorphological analysis considering the intricate resemblance it shares with other tumors originating from eccrine glands. Poroid hidradenoma has only recently been recognized, and only a limited number of cases have been reported in the medical literature. In this instance, we present an unusual occurrence of a giant poroid hidradenoma on the left forearm of an elderly patient.

Gastric Adenocarcinoma With Unusual Clear Tubulopapillary Morphology: A Case Report.

Gastric clear cell adenocarcinoma is an extremely rare variant of papillary adenocarcinoma of the stomach. It is associated with a poor prognosis due to its frequent lymphovascular invasion and also its higher risk of recurrence. It is characterized morphologically by a clear appearance of tumor cells, which can be easily confused with a metastasis of a clear cell carcinoma, particularly of renal origin. Very few cases have been previously reported in the literature, which makes it a very poorly known variant. Here, we report the case of a 64-year-old patient who presented with a polypoid lesion in the pylorus, revealed by epigastric pain and chronic vomiting. Histological analysis showed a gastric clear cell adenocarcinoma with a tubulopapillary architecture. Immunohistochemical examination excluded a metastasis of renal origin. Through this case report, we highlight the importance of recognizing such an uncommon and unusual variant of gastric adenocarcinoma, to prevent any potential misdiagnosis.

Solitary Necrotic Nodule of the Liver: A Benign Mimicker of Malignancy.

Solitary necrotic nodule (SNN) of the liver is an uncommon and benign finding in liver pathology. Typically, it appears as a single and asymptomatic lesion, primarily located at the subcapsular region of the right lobe of the liver. Unfortunately, it is easy to mistake this benign lesion for a primary or secondary neoplastic lesion, making it a potential diagnosis pitfall for liver malignancies. The diagnosis of SNN can be difficult to determine as the imaging findings frequently lack specificity. This brings out the importance of histomorphological examination to accurately identify this lesion, and to rule out any possible malignancies. We report here the case of a 35-year-old woman with a history of squamous cell carcinoma of the cervix, who presented a solitary nodule on her liver that was falsely diagnosed as a metastatic lesion in the liver at imagery. The aim of this article is to highlight the importance of using special stains and immunohistochemical staining for diagnosing SNN and excluding any necrotic metastases of the liver. We demonstrated that the absence of a reticulin meshwork in the necrotic core should prompt consideration of a necrotic metastasis in the liver, rather than a solitary necrotic nodule.

Nodular Basal Cell Carcinoma in an Unusual Groin Location: A Rare Presentation.

Basal cell carcinoma is the most frequent skin malignancy with a constant rise in its incidence. It affects typically the head and neck of elderly patients. However, the literature in English shows its occurrence in many uncommon locations. In our work, we report a case of basal cell carcinoma occurring in the groin region in a 66-year-old male patient, with no particular medical history. We also discuss through a literature review, the characteristics of this common neoplasm when it occurs in the groin and in other atypical locations.

Two cases of Dieulafoy's lesion in unusual sites: A rare case reports of gastrointestinal bleeding.

INTRODUCTION: Dieulafoy's lesion (DL) is a vascular malformation that can lead to massive gastrointestinal bleeding. It's usually found in the stomach. However, DL's occurrence in atypical sites such as the small bowel and colon is exceptionally rare, posing significant management challenges. CASE PRESENTATION: In this report, we present two cases of DL occurring in uncommon sites, each managed with distinct approaches. Case 1 is a 50-year-old man admitted to the emergency department due to massive GI bleeding and hemodynamic instability. The diagnosis of DL was established through computed tomography angiography and confirmed by histopathological examination after emergency surgery. Case 2 involves a 68-year-old woman presented with melena due to a colonic DL. This case was successfully managed through an endoscopic hemostasis approach. DISCUSSION: Dieulafoy's lesions (DL) were first identified as a large submucosal artery lacking typical gastric ulcer characteristics in three of Paul Georges Dieulafoy's patients. This lesion is responsible for approximately 1-2 % of all cases of gastrointestinal bleeding. Endoscopy is the preferred method for diagnosing and managing DL lesions, especially in cases of active bleeding that is accessible. However, if endoscopic treatment or angiographic embolization fails, a surgical approach may be needed. CONCLUSION: DL presents a diagnostic challenge due to its rarity and is not usually included in the differential diagnosis of gastrointestinal bleeding, particularly when occurring in unusual sites. Endoscopy is the preferred method to identify DL and a possible therapeutic approach in active bleeding. However, if endoscopy hemostasis fails, angiographic embolization or surgical intervention may be required.

Pseudoangiomatous stromal hyperplasia: a rare cause of gynecomastia in men.

A 17-year-old male with chest malformation and left breast enlargement underwent surgery for gynecomastia. Histological examination revealed mammary fibrous stroma with ductal hyperplasia and features of pseudoangiomatous stromal hyperplasia. Postoperative follow-up showed no complications, but 8 months later, the patient experienced a mild recurrence with enlargement of the nipple-areolar complex. Although recommended for secondary glandular resection, the patient declined further surgery.

Bladder papilloma: A rare benign tumor - Case report and literature review.

INTRODUCTION AND IMPORTANCE: Bladder papilloma, a rare benign tumor of the urinary tract, accounts for 1-4 % of bladder tumors. Its distinct features, diagnosed through light microscopy, include architectural and cytological characteristics. Despite its rarity, bladder papilloma is clinically significant due to its distinct traits, low recurrence risk, and potential progression to other urothelial neoplasms. Understanding this condition is crucial for early diagnosis and optimal patient care. CASE PRESENTATION: A 66-year-old male with benign prostatic hyperplasia presented with one month of intermittent hematuria. Physical examination and laboratory tests were unremarkable. Imaging revealed an 11 × 10 × 7 mm echogenic nodular lesion with calcifications on the right bladder wall. Cystoscopy identified a polypoid lesion, leading to transurethral resection. Histopathological examination confirmed bladder papilloma without malignant features. CLINICAL DISCUSSION: Bladder papilloma typically presents with hematuria, mainly in younger patients, with low recurrence and rare progression to aggressive cancers. Diagnosis involves endoscopy and resection, followed by cystoscopic surveillance. Understanding its classification, characteristics, and risk factors aids in accurate management. CONCLUSION: Bladder papilloma diagnosis relies on light microscopy, with hematuria as a primary symptom, often in younger patients. Recurrence risk is low, and progression to aggressive cancers is rare. Diagnosis involves endoscopy, resection, and cystoscopic follow-up. This case enhances our understanding of bladder papilloma, contributing to improved care.

Multimodal Therapy of Locally-Advanced Penile Cancer: A Case Report With Literature Review.

Cancer of the penis is a rare tumor that occurs in the elderly. Because of its rarity, it is often not diagnosed early, and its treatment poses difficulties for practicing oncologists. We report the case of an elderly patient treated for locally advanced squamous cell carcinoma (SCC) of the penis, with a review of the literature. A 71-year-old man, who had been complaining of pruritus on the penis two years ago, presented with an ulcerated lesion on the prepuce and the glans. A biopsy of the lesion with pathological study showed a SCC of the penis. Pelvic MRI showed tumor thickening centered on the glans of the penis, infiltrating the fascia and the spongy urethra with discrete upstream dilatation and bilateral inguinal adenomegaly. CT scan of the neck, chest, abdomen, and pelvis showed no secondary localizations. Treatment initially consisted of carcinological surgery by a partial penectomy with bilateral inguinal lymph node dissection. The tumor was therefore classified as pT3N3M0. A PET CT scan performed later was in favor of local and regional recurrence. Surgery was not feasible, so concomitant chemo-radiotherapy was indicated at a total dose of 70 Gy in 35 fractions of 2 Gy concomitantly with platinum-based chemotherapy, withgood evolution.

A Rare Case of Trichobezoar Revealing Undiagnosed Celiac Disease.

Trichobezoar is a relatively rare condition in children, mainly observed in young girls with psychiatric disorders. While documented cases of trichobezoar associated with celiac disease exist, such occurrences remain uncommon in the literature. The association between the two can be explained either by behavioral disorders resulting from a deficiency in iron and folic acid or directly by celiac disease. Treatment is predominantly surgical, and psychological support plays a crucial role in preventing the likelihood of recurrence. We present an unusual case involving the discovery of gastric trichobezoar in a 15-year-old girl who had undiagnosed celiac disease. The condition manifested after she experienced abdominal pain and pallor.

Unusual Metastasis of Gastric Signet Ring Cell Carcinoma to the Breast: A Case Report of a Young Moroccan Patient.

Although gastric cancer is known to be an aggressive tumor that can spread throughout the body, breast metastases are uncommon. This entity is rarely reported in the literature, with an estimated incidence of 0.5 to 1.3%. We report a case of a rare association between a gastric subtype of signet ring cell carcinoma and metastasis to the breast. This uncommon situation is only documented through case reports. Most breast metastases have been detected after diagnosis of primary gastric cancer, during the first year. Several risk factors have been suggested to explain the aggressive behavior of these tumors, which correlates with very poor prognosis and short survival. We report the case of a 22-year-old female patient presenting with widespread metastatic gastric signet ring cell carcinoma with an unusual secondary site in the breast. The diagnosis was confirmed by immunohistochemistry (IHC) and radiology, and the patient was treated with palliative chemotherapy in accordance with the decision of the multidisciplinary tumor board.